Derm Summary Table.pdf

KEY WORDS 1) Structures and function of the skin

abnormal pathologies in the epidermis: - parakeratosis- nucleus retention in the s.corneum - acanthosis- thickening of the skin due to hyperplasiaa of the Malphigian layer (s.basale +s.spinosum)

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2) Primary lesions

- definition: Primary lesions are the initial manifestation of the -

3) Secondary lesions

epidermis, dermis, subcutaneos tissue function of the skin thick vs thin skin (stratum lucidum in thick skin only) layers of the epidermis layers of the dermis: reticular vs. papillary (coolgen, elastin, blood supply, nerves) Langer’s lines: due to collagen and elastic fibers arrangement in reticular dermis. importance for scarring. cells of the epidermis: keratinocites, melanocytes, Langerhans cells, Merkel’s cells (sensation role), Meissners (light touch) and Pacini (pressure and vibration) corpuscles in the dermis Hair follicle: 4 parts: bulb, suprabulb, istmus, infundibulum. structure: dermal papillae, inner+outer root sheats, sebaceous gland, arrectot pili, hair shaft Glands: sweat glands (merocrine secretion), sabecous glands (apocrine secretion) Nail: eponichyium (cuticle), hyponichium, nail plate, nail bed, nail root, lunula (visible part of the root of the nail), perionichium disease, unaffected by the course of the disease or manipulation by the patient lesions: macule, patch, papule, plaque, nodule, bullae, vesicale, urtica, pustule, tumor, urtica/wheal examples to each of them > 1 or <1 cm correspondly papule and nodule: the role of depth in dermis examples for different types of macule: hyper/hypopigmentes, vascular, exogenous epidermal/subepidermal location for vesicles and bullae

- 12 lesions: scale, crust, erosion, excoriation, ulcer, gangrene, -

fissure, atrophy, lichenification, poikiloderma, sclerosis, scar, calcinosis types of scales: fine, silver, dry + examples erosion vs. excoriation: Erosion= depressed circumscribed and moist or crusted lesion from focal loss of all or part of the epidermis Excoriation= a linear (line-like) erosion resulting from scratching. scale= Abnormal accumulation of corneocytes, secondary to excessive epidermal turnover calcinosis: hard calcium containing nodules or plaques

KEY WORDS 4) Urticaria, angioedema, anaphylaxis

urticaria

- definition : Elevated primary lesion that is caused by leakage of fluid from a vessel

- acute (few days, <6 weeks) vs. chronic urticaria (>6 weeks ) - pathophysiology of urticaria - 3 forms of urticaria: 1. allergic- Hymenoptera toxins, penicillins and some food stuffs 2. pseudo allergic urticaria - histamin release but no IgE, Chronic urticarial is likely to be pseudoallergic 3. physical urticaria- solar urticaria, dermographism, cold and hot urticaria, cholinergic urticaria, pressure urticaria

- diagnosis (prick, LTT) and treatment of acute urticaria angioedema

- definition: the rapid swelling (edema) of the dermis, subcutaneous tissue, mucosa and submucosal tissues. It is very similar to urticaria, but urticaria, commonly known as hives, occurs in the upper dermis - causes: allergic, drug-induced, hereditary - drug induced: ACE-inhibitors (ACE normally degrades bradykinin) - hereditary: C1-esterase inhibitor deficiency (C1INH) normally: - C1INH (C1 esterase inhibitor) —> inhibit bradykinin and kalikrein - kalikrein —> inhibits the association of C1r and C1s with C1q to prevent the formation of the C1-complex which - in turn activates other proteins of the complement system - in hereditary form: C1INH deficiency —> no kalikrein inhibition —> continuous complement activation and no bradykinin inhibition —> high bradikynin —> vasodilation 5) Eczemaz (allergic contact dermatitis, irritant contact dermatitis, dyshidrosiform and microbial eczema)

- eczema definition: Heterogenous group of non-infectious inflammatory dermatoses

- acute vs. chronic eczema features - allergic contact dermatitis: erythema, blisters, itchy, rash. type -

microbial/nummular/discoid eczema:

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4 HSR, causes, most frequent locations, treatment, diagnosis —> patch test is the gold standard irritant contact dermatitis: Direct cytotoxic effect of a chemical or physical agent (single, or repeated application) on the skin causing inflammatory reaction without immunological mechanisms irritants examples, skin features pompholyx: blisters, side of the fingers, mostly idiopathic nummular eczema: describe the lesion: coin-shaped, round or oval-shaped itchy lesions, recurrent and chronic, sarcoidosis

KEY WORDS 6) Topical dermatological treatment

- Topical preparations can be: Protectans (absorbents), antiinfective agents, anti-inflammatory agents (corticosteroids), emollients (skin softeners), keratolytic agents

- steps between application of the topical agent and its local LAAPPR RBUPPU

action: LAAPPR (liberation, adsorption, absorption, penetration, permeation, resorption) (release—>binding—>uptake—>passage—>passage—>uptake) • vehicles types: 8 creams (2 forms: oil in water and water in oil), lotion, solution (e.g., antiseptic/antibacterial solution), ointment, gels, paste, shake lotion, powders • active ingredients: 6 - corticosteroids: uses, contraindications, SE, 4 categories (Niedner classification): I = weak (hydrocortisone, prednisolone) II = moderate (hydrocortisone butyrate, triamcinolone)
 III = strong (betamethasone, mometasone, flucinolone acetonide) IV = very strong (clobetasol)

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topical calcineurin inhibitors (tacrolimus, pimercrolimus) topical NSAIDS (diclofenac, HA) topical antibiotics (mupirocin) topical antivirals (acyclovir) topical retinoids (isotretinoin). teratogemic!!

KEY WORDS 7) Sarcoidosis, Granuloma annulare, TB of the skin, leprosy

• sarcoidosis: - Sarcoidosis is a systemic disease that features non-caseating granulomas. Its triggered by IFN-γ producing TH1-cells - role of other organs involve (lungs, heart, liver, eye, nervous sys) - forms of sarcoidosis of the skin: 1) specific lesions: lupus pernio, scar sarcoidosis, Maculopapuplar eruptions, skin plaques 2) non-specific lesions: erythema nodosum, erythema multiforme, nummular eczema, calcinosis cutis , pruritus

localized GA:

* lupus pernio: reddish-purple plaques and nodules on the nose, cheeks, lips, ears and digits. Lesions may ulcerate and later heal with telangiectatic scars.

generalized GA:

• granuloma annulare: - blue-pink papules surrounding an area of central atrophy - Granuloma annulare is a common benign granulomatous disease of the skin and subcutis with an unknown etiology. Probably type 4 HSR. - associated with DM - localized form: common location: skin over joints, knuckles - generalized form • TB of the skin: - main types: 1) PIT- primary inoculation TB: chancre, non-immunized host, direct inoculation 2) TB verrurcosa cutis (TVC)- warty, crusted plaque, previously infected, direct inoculation 3) Scrofuloderma- ulcer, sinus tract 4) Lupus vulgaris- reddish-brown plaque, apple-jelly nodules on diascopy 5) Miliary TB- exanthem, macule and papule 6) Tuberculid- generalized exanthem, erythema induratum (Bazin disease- recurring nodules or lumps) • Leprosy: - M. laprae: nerve invasion and multiplication, intracellular - types: mildest tuberculoid form to the most severe lepromatous leprosy 1) Tuberculoid- benign, macular skin lesion, thickened superficial nerves, hypo pigmented hypesethtic macule, positive lepromin skin test (CMI associated) 2) borderline tuberculoid 3) borderlines-borderline 4) borderline-lepromatous 5) lepromatous-malignant, nodular raised skin lesions (lion face)
 - negative lepromin skin test (poor CMI), diffuse skin infiltration, multiple nodular lesions, sensory loss

KEY WORDS 8) Drug eruptions (type1-4 HSR)

- classification: type A (predictable, strictly dose dependent) vs. type b reactions (not-predictable, usually not dose dependent)

- talk about the different types of HSR reactions in terms of: onset,

Severe Cutaneous Adverse Reactions (SCAR) to drugs include:

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mechanism, symptoms and common drugs that can cause each one of them type 1: peniciilins type 2: penicillins, sulfonamide, isoniazid tupe 3: rituximab. immunoglobulins type 4: sulfametoxazol, anticonvulsants

common and unique drug reactions:

1) Drug hypersensitivity response DRESS (type 4 HSR) (anti-gout, anti-epileptics) 2) Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN) (penicillin, sulfonamides, antiepileptics) 3) Acute Generalized Exanthemateous Pustulosis (AGEP) (penicillin) 4) Exfoliative dermatitis/drug induced erythroderma

9) phototherapy and photo chemotherapy

- common drug eruptions: 1. maculopapular exanthema (penicillin), 2.fixed drug eruption (tetracycline, metronidazole), 3. urticaria and angioedema (ACE-inhibitors)

- unique drug eruptions: 1. acneiform and rosacea-like (OC, corticosteroids), 2. acute generalized exanthematous pustulosis =AGEP (penicillin), 3. allergic contact dermatitisbaboon syndrome (antihistamine) 4. hyper pigmentation (ACTH, estrogens, antimalarials), 5. hypersensitivity syndrome=DRESS (carbamezapine) 6. lichen-planes-like (beta blockers) 7. lupus erythematous (procainamide) 8. photo allergy and phytotoxicity (nalidixic acid, NSAIDs) 9. hypertrichosis (minoxidil) phototherapy:

- Phototherapy usually involves UVA (320-400nm) or UVB (280-320nm) light

- general uses: psoriasis, atopic dermatitis, early mycosis fungicides

- Fluorescent and high-pressure lamps are used to deliver UVA and UVB

- sources: UVB311nm tubes and UVA1 high pressure lump - UVB: psoriasis, atopic dermatitis, mycosis fungoides, -

vitiligo, 5 times weekly, minimal erythema dose (MED)= Minimal dose that causes visible slight erythema UVA1: anti-inflammatory, anti-sclerotic —> used for systemic sclerosis and morphea

photochemotherapy:

- using psoralen with UVA—> PUVA - The peak serum and skin level is from one to 6 hours after ingestion.

- uses: Psoriasis, mycosis fungoides, atopic dermatitis, vitiligo, lichen planus, scleroderma, GVHD and polymorphic light eruption - 3-4 times weekly - MPD- minimal phytotoxic dose - SCC risk from PUVA * psoralens: group of natural furocoumarins, commercially derived from Ammi Majus, a plant found in Egypt. They are present in celery, carrots, parsley, parsnip and other vegetables

KEY WORDS 10) Fungal infections of the skin (superficial and deep mycosis, pytriasis versicolor)

what is Tinea? Tinea, often called ringworm, is any of a variety of skin mycoses.Tinea is a very common fungal infection of the skin. Tinea is often called "ringworm" because the rash is circular, with a ringlike appearance. superficial mycoses:

- 2 causes: Dermatophytes – multicellular filaments or hyphae or Yeasts – unicellular forms that replicate by budding

- microscopic exam (KOH mount) and culture (Saburados dextrose agar, dermatophyte tes medium DTM)

- dermatophyte infection=tinea infections: typical for tinea is annular spreading erythema with small papules and pustules. Scale is prominent at the advancing edge of the lesion. - They can be spread from humans (antrhophilic), animals (zoophilic) or soil (geophilic). Zoophiclic infections produce more inflammation than antrhrophilic. - 3 main genera: Trichophyton (nails+hair+skin)m Epidermophyron (skin+nail) microsporum (hair+skin) - main types of infections: 1) Tinea corporis: body, M.canis, lesions are circular and sharply marginated with a raised edge (“bull’s eye” appearance) 2) Tinea cruris: groin, T. rubrum, itchy 3) Tinea pedis (athlete’s foot)- intensely pruritic lesions, T. rubrum, T. interdigitale, most common, interdigital area of the foot 4) T.manus 5) Tinea Faciei- T. rubrum, plaque or annular 6) Tinea barbae- Trichophyton mentagrophytes and T. verrucosum, folliculitis - DD - oral vs. topical azoles (e.g. ketoconazole, clotrimazole) - and allylamines (e.g. naftifine, terbinafine) 


- treatment rose gardener’s disease:

Deep mycoses:

- These infections can be divided into subcutaneous and systemic mycosis. And they are more common in the tropical regions of the world. - subcutaneous mycosis: Sporotrichosis (Rose gardner’s disease) --> Nodules appear under the skin along the lymphatic channels - systemic mycoses: immunocompromised. mainly erythematous papules, ulcerated: c.neoformans, blastomyces, cocidiodes imitis, histoplasma capsulatum - treatment: systemic voriconazole, itraconazole, amphotericin B

Pytriasis (tinea) versicilor: - not caused dermatophyte! by a yeast! - summer, tropics, Malasazia furur, brown 0.5-1 cm macules, spaghetti & meatballs under the microscope with KOH mount

KEY WORDS 11) fungal infection of the nail, scalp and mucous

favus:

fungal infection of the nail: Tinea unguinum/onychomycosis :

- T. rubrum mainly - risk factors - 4 clinical subtypes: 1) 2) 3) 4)

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distal subungual proximal subungual white superficial dystrophic treatment: nail polish, systemic: imidazle/terbinafine

Tinea capitis:

- M.canis, T.verrucosa - manifestations: 1) 2) 3) 4) kerion

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noninflammatory infection “black Dot” tinea Capitis (broken of hair near the scalp), Kerion (boggy, purulent inflamed nodules and plaques), Favus (=honeycomb, yellow adherent crusts, atrophy and scarring—> scarring alopecia) T. verucossa: Kerion topical (imidazole) and systemic (griseofulvin, itroconazole and terbinefine)

candidiasis:

- candida albicans mainly - oral candidiasis (white oral thrush pseudomembrane), vulvovaginal vaginitis (cheesy white discharge), candidate balanitis (erythema, erosions and white patches on the glans and under foreskin )

12) dermatologic surgeries and aesthetic procedures

- biopsy: shave, punch, elliptical excision - superficial removal: curettage with a ring curette, scissor excision

- excisions: with safety margins– standard of tumor removal - plastic reconstructive techniques: 1) skin flap 2) skin graft (for face- full thickness graft, post auricular is the best source) 3) split thickness skin graft (only part of the skin- the epidermis in contrast to full thickness. larger areas should be covered, taken from the buttock) 4) mesh skin graft (burn patient macaroni machine) 5) skin substituents (burn patients, Cultured keratinocytes from the patient on an artificial dermal meshwork ) - other: circumcision, nail operation - aesthetic: botulinum, fillers (resorbable and non-resorbable, hyaluronic acid, collagen, calcium hydroxyl apatite), peeling (superficial, medium, deep), lifting, liposuction, cosmetic vein surgery, plastic reconstructive surgery

KEY WORDS 13) Acne, rosacea, perioral dermatitis, seborrheic dermatitis

Acne:

- a disease of the pilosebaceous follicles and therefore affects the face and upper trunk.

- risk factors: genetics, androgens - primary (papules, pustules, cysts) and secondary (scars) lesions

conglobata: comedones, papules, pustules, nodules, cysts, and multichanneled draining sinuses. Scarring and keloid formation are frequent sequelae cystica: comedones, papules, indurated painful nodules and pustules with haemorrhagic crusts

- 4 grades - types of acne: acne comedonica (grade 1) / papulopustulosa -

(grade 2) / conglobata (grade 3) / cystica (grade 3) propionibacterium acne relation to the hair follicle and sebaceous gland comedo: black vs white (open vs. close) dd: rosacea, folliculitis, perioral dermatitis treatment: topical and systemic antibiotics (erythromycin), retinoids (isotretinoin- !!‫ רקוטאן‬teratogemic!), phototherapy

Rosecea:

- the “acne” of the old- but no comedos, whiteheads or nodules! - linked with dysregulation of facial blood flow, fair skin color, Dermodex mites, hereditary oily skin, excess ingestion of alcohol, hot drinks and spicy foods, topical or systemic corticosteroids. - primary lesions: papules, pustules, diffuse redness, telangiectasia - secondary lesions: bulbous nose- rhinopehyma - main subtypes: 1) rhynophyma (due to sebaceous hyperplasia) 2) erythemato-telangiactatic 3) ocular rosacea 4) papulopustular - treatment: topical (metronidazol), systemic (tetracyclin), sun protection - never use topical steroids!!! perioral dermatitis:

- grouped papules around the mouth (with a pale spared zone adjacent to vermilion) or around the eye.

- cause—> mainly due to topical steroids seborrheic derm:

- Sharply bordered erythema with greasy yellow scales are -

always present. (salmon-pink, thin scaly and ill defined plaques) mainly scalp, and other high sabeceous-gland-rich areas, blepharitis peaks in infants and elderly Etiology is dependent on 3 factors, sebum, Malassezia yeast, and individual susceptibility. treatment: keratolitics (salycilic acid), anti fungal (ketoconazole), ketoconazole shampoo

KEY WORDS 14) Lyme disease, scabies, pediculosis acrodermatitis chronica atrpicans

Lyme disease: borrelia borgdirferi ixodes ricinus tick and its removal diagnosis: ELISA and immunoblotting is the method of choice 3 stages: 1) localized lyme disease (3-33 days after tick bite)- erythema chronicum migrans, flu-like illness 2) early disseminated lyme disease (days-weeks)- multiple erythema migraines, early neuroboreliosis (bells palsy, aseptic meningitis) 3) late lyme disease (months-years)- lyme arthritis, acrodermatitis chronic atropicans (unilateral violet discolouration of the extensor parts of the upper or lower limbs, especially the dorsum of the hand, elbow, instep, ankle or knee, and skin atrophy)

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scabies:

- lesions: pruritic erythematous papules and excoriations -

(Papules develop as an allergic type 4 HSR reaction to the mite feces) mite: sarcoptes scabei risk factors intense pruritus, night mainly, interdigital folds, palms, wrist, penis, nipple burrows/tracks permethrin

pediculosis:

- louse - 3 subcategories: pediculosis capitis (head louse), pediculosis corporis (body louse), phthiriasis (pubic louse, STD!!)

- permethrin

KEY WORDS 15) bacterial infections of the skin (impetigo, ecthyma, paronychia, folliculitis, furuncle, carbuncle, anthrax)

- skin defense mechanisms: ph, innate+adaptive immune, continuous desquamation

- factors favoring bacterial growth impetigo: - stap, and beta-hemolytic strep (non-bullous impetigo) - types: superficial (non-bullous), ecthyma and bullous: 1) superficial (non-bullous): - staph+ strep - lesions: macules that turn into vesicles & papules/pustules and then into crusted lesions, no scar - invade site of minor trauma 2) ecthyma: - usually strep. - like non-bullous, but then into necrotic ulcer, scar!! 3) bullous: - staph exfoliatin toxin - lesions: bullae and crusts, no scar furuncle & carbuncle:

- pus filled tender nodule, staph aureus - centered on hair follicle—> it is a deep form of bacterial folliculitis!!

- multiple confluent heads = carbuncle folliculitis:

- superficial or deep (acne is a variant of folliculitis) - stap aureus, malassezia, tinea capitis - pseudofolliculitis (regrowing hair) paronchia:

- Painful infection of the nail fold caused by S.aureus due to damaged protective cuticle Anthrax

- bacillus anthracis, zoonosis, spores entering the skin - cutaneous anthrax: “malignant pustule”=painless bump —> blister—> ulcerate, necrotic eschar

- swollen lymph nodes —> lymphangitis

KEY WORDS 16) superficial thrombophlebitis, chronic venous insufficiency

- venous circulation principles: 1) superficial suprafacial veins (great saphenous-GSV, short saphenous, dorsals pedis), 2) deep sub facial veins (common femoral, superficial femoralSFV, anterior+posterior tibial, popliteal), 3) perforator veins (Dodd- GSV-SFV, Boyd & Crokett- GSVpost. tibial) superficial thrombophlebitis:

- Superficial thrombophlebitis is an inflammation of a superficial vein usually due to infection or trauma from needles and catheters with partial or complete thrombotic closure of the lumen. - pathogenesis: trauma to the superficial vein (e.g., catheter) —> damaged endothelium —> inflammation +/- thrombus formation - causes/predisposition: Virchow’s triad (venous stasis, hypercoagulability, and endothelial trauma) —> inflammation/ blood clot form. - complications (clot moves into deep veins —> DVT) - high risk patients (DVT history, varicose veins, IV injections, blood clotting abnormal., cancer, smoking, stasis e.g., long air flights) - signs and symptoms: swollen, red leg, cyanotic, painful - dd, diagnosis (clinically+history)
 Chronic venous insuff:

- Chronic venous insufficiency results from failure of centripetal return of venous blood and increased capillary pressure

- pathogenesis: initial event (e.g., defect valve) —> aggravation

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atrophie blanche:

of venous stasis and varicose vein dilatation —> thrombosis —> fibrin is deposited in the extravascular space undergoes organization causing lipodermatosclerosis and obliteration of lymphatics and microvasculature —> stasis dermatitis —> ulceration the resultant changes include: edema, stasis, dermatitis, hyperpigmentation, fibrosis of the skin and the subcutaneous tissue (lipodermatosclerosis), atrophie blanche, corona phlebetetica paraplantaris, varicose veins of the leg, and ulceration. causes: usually congenital damage of superficial or less commonly deep veins valve—> reflux, damaged perforator veins, —> blood flows from deep veins into superficial venous plexus, thrombus formation aggravating factors: pregnancy, increased CO lipodermatosclerosis—> “champagne bottle” due to edema above and below the sclerotic region
 - CAEP staging system (clinical picture, anatomy, etiology, pathophysiology) examination: doppler US, doppler ankle-brachial index (normal Pleg/Parm = 1 )

KEY WORDS 17) skin and internal diseases: PNS, pruritus

Pellagra:

scurvy:

internal disease and skin symptoms: 1. hormonal changes and disorders: pregnancy: striae distensae, polymorphic eruption of pregnancy (PEP), pustuler psoriasis 2. disorders of the adrenal: Cushing (high cortisol): moon face, Addison’s disease (lack of GC): brown hyperpigmentation 3. disorders of thyroid gland: Graves’ disease: dermopathy, hypothyroidism: myxedema) 4. liver diseases: jaundice, hemochromatosis (iron overload), Wilson’s disease (Yellowish discoloration of skin and eyes (jaundice)). 5. metabolism and nutrition disorders: xanthomas, xanthelasma 6. neuropsychiatric diseases: neuroses (excoriations), psychoses (trichotillomania) 7. skin disease associated with DM 8. vitamin D deficiency: Scurvy (petechia and hemorrhage) 9. niacin deficiency: Pellagra (3D’s: dermatitis, dementia, diarrhea)

PNS:

- definition: clinical syndrome which is the consequence of cancer

Bazaex syndrome:

in the body but that, unlike mass effect, is not due to the local presence of cancer cells but due to an altered immune system response to a neoplasm. - obligate PNS markers: 1. acanthosis nigricans (GI + GU adenocarcinoma)- darkening (hyperpigmentation) and thickening (hyperkeratosis) of the skin, occurring mainly in the folds of the skin in the armpit (axilla), groin and back of the neck. 2. acrokeratosis neoplastica=Bazex syndrome (Hodgkin lymphoma, SCC of URT/GI): - Severely abnormal nails with painful paronychia (swollen red nail folds) with no evidence of bacterial or fungal infection. - Scaly eruptions on the external ears that may progress to involve the cheeks and nose - Diffuse thickening and scaling of the palms and soles (keratoderma), with a peculiar honeycomb appearance - Eventually the skin disease may spread to involve trunk, limbs and scalp 3. hypertrichosis lanuginose acquistia (colorectal, breast, lung)

- facultative PNS markers: 1. dermatomyositis 2. generalized pruritus 3. Leser-Trelat syndrome- abrupt appearance of multiple seborrhoeic keratoses that rapidly increase in their size and number.(lung & GI adenocar.) 4. thrombosis, panniculitis, thrombophlebitis pruritus due to internal diseases (DM, malignancies, end-stage renal disease, biliary cirrhosis)

KEY WORDS 18) ulcus cruis (leg ulcers)

Leg ulcers refer to full thickness skin loss on the leg or foot due to any cause. They occur in association with a range of disease processes, most commonly with blood circulation diseases. Leg ulcers may be acute or chronic. Acute ulcers are sometimes defined as those that follow the normal phases of healing; they are expected to show signs of healing in less than 4 weeks and include traumatic and postoperative wounds. Chronic ulcers are those that persist for longer than 4 weeks and are often of complex poorly understood origin.

1) venous origin: most common cause of leg ulcer! - Ulceration of the lower leg due to CVI, DVT, phlebitis, previous leg injury - most common site—> medial lower calf- especially over the malleolus! - clinical present: swollen, heavy, hyper pigmented - complications (infections, lymphedema), diagnosis (no pulse, doppler) - treatment (compression=gold standard! wound care)

2) arterial origin: account for 10% of leg ulcer

- pathogenesis: occlusion (atherosclerosis) —> ischemia —> necrosis

- risk factors (diet, smoking, DM, HTN) - clinical presentation: no pulse!!, distal, claudication, sharp edge, shiny

- diagnosis (ABI<0.8, doppler, ESR) - manage: no compression!! anticoag., vascular reconstraction, vasodilators, diet, stop smoke) diabetic foot —> callus! 3) neuropathic ulcer: diabetic patient - no pain, pressure points, amputation, callus

KEY WORDS 19) atopic dermatitis Fillagrin role: Recently, there is emerging evidence that inflammation in atopic dermatitis results primarily from inherited abnormalities in the skin – the skin “barrier defect”. This barrier failure causes increased permeability of the skin and reduces its antimicrobial function. An inherited abnormality in filaggrin expression is now considered a primary cause of disordered barrier function. Filaggrins are filament-associated proteins which bind to keratin fibres in the epidermal cells. The gene for filaggrin resides on Chromosome 1 (1q21.3). This gene was first identified as the gene involved in ichthyosis vulgaris. It is postulated that the loss of filaggrin results in: ▪ Corneocyte deformation (flattening of surface skin cells), which disrupts the organisation of the extracellular lipid (fat) – the lamellar bilayers. ▪ A reduction in natural moisturising factors, which include metabolites of pro-filaggrin. ▪ An increase in skin pH which encourages serine protease activity – these are enzymes which digest lipid-processing enzymes and the proteins that hold epidermal cells together. Serine proteases also generate active cytokines like IL-1a and Il-1beta and promote skin inflammation.

- Atopic dermatitis is a chronic pruritic inflammation of the epidermis and dermis, often associated with a personal or familial history of asthma, allergic rhinitis or conjunctivitis (i.e., atopic tendency) - lesions: dry skin and pruritus, consequent rubbing leas to increased inflammation and lichenification and to farther itching and scaring; ITCH-SCRATCH cycle!! - peak incidents (3-24 months- 15-20%=> most common prevalence peak in early childhood!!), 6-16 years, 20-30 years, >60) - pathogenesis: complex interaction of environmental+genetical +immunological factors: - defect barrier function and fillagrin role - staph. aureus infection - altered immunity -unique to this type of dermatitis —> high IgE — type 1 HSR! - 4 criteria of atopic dermatitis: 1. itching 2. specific morphology 3. chronic symptoms 4. atopic anamnesis

- localization: 1) babies: napkin area not involved, face=most common site!! usually start on the face and later the whole body can be involved 2) children- flexural, neck 3) adults: hand, feet, flexures - diagnosis (clinicaly, IgE, (+) prick test) - complications - treatment (preventive, other)

KEY WORDS 20) syphilis

- Treponema pallidum spirichette - transmission (STD, needle, blood trans.) - stages: 1) primary syphillis: after 3 weeks, at infection site, single papule that ulcerate —> chancre (painless chancre, unnoticed, heals spont.), symmetric regional lymphadenopathy (bubo indolens) (50% of patients are negative by nonspecific serology) 2) secondary syphillis: 9 weeks later, widespread maculopapular rash on the palms and soles and “flu-like” symptoms, very infectious, unilateral tonsillitis, condylomata lata (grey-white moist plaque full with spirochette), opaline plaque (in the mouth), patchy alopecia (beard, hair, diffused), lymphadenomegaly (both specific and non-specific serology are positive) 3) latent period: (early- infectious, late- not infectious) (positive) 4) tertiary syphillis: 3-10 years later, gummas (sollitary subcut. granulomatous lesions in CNS), neurosyphillis, spinal cord disease, aneurysms (non-specific may be negative) 5) congenital syphilis: tabes dorsalis, Hutchinson teeth - diagnosis: • darkfield microscopy • specific (treponemal)=confirmatory test (FTA-ABS or TPHA, using treponema Ag to react with patient’s serum Ab) • non-specific (non-treponemal) serologic tests=screening tests (VDRL&RPR: cardiolipin demonstrate reagin= mixed IgM and IgG due to cross reaction) SCREENING —> than CONFIRMING - treatment: penicillin. look at the minimal!

21) psoriasis

- a complex, multifactorial disease that appears to be influenced by genetic and immune-mediated components.

- pathogenesis- not completely understood. Multiple theories exist

Types: 1) guttate=droplet-like 2) vulgaris 3) chronic plaque psoriasis (80-90%) 4) inverse=thin plaque mainly in the axillary & inguinal folds 5) palmoplantar 6) pustular (5%) - sterile pustule!! 7) nail psoriasis 8) psoriatic arthritis - hand mainly 9) psoriatic erythroderma=> whole body

regarding triggers of the disease process: infectious episode, traumatic insult (Kobner phenomenon), and stressful life event. In many patients, no obvious trigger exists at all. However, once triggered, there appears to be substantial leukocyte recruitment to the dermis and epidermis resulting in the characteristic psoriatic plaques. Specifically, the epidermis is infiltrated by a large number of activated T cells, which appear to be capable of inducing keratinocyte proliferation. - epidemiology: 2 types 1) type 1 (<40, family history, HLA B13) 2) type 2 (>40, no family history, B27) - histology: acanthosis (epidermal hyperplasia), parakeratosis (nucleus retention) Munro microabscesses (NT collection in S.corneum), thinning of the epidermis above elongated dermal papillae —> bleeding when scale picked up= Autspits sign! - lesion: sharply defined erythematous silvery-scale plaque - treatment (PUVA, topical steroids, topical retinoids, calcineurin inhibitors, immunosupressors, biological agents, coal tar) DONT USE SYSTEMIC STEROIDS!!!

KEY WORDS 22) Sexually transmitted infections (incl. gonorrhea, urethritis non gonorrhoica, other venereal diseases)

neisseria gonorrhea

- intracellular diplococci on gram - ceftriaxone gonorrhea: male (uretritis, proctitis), female (PID, endocervicitis, uretritis, chronic gonorrhea), infant (opthlmia neonatorum), disseminated. ascending exudate=>more purulent than chlamidya’s exudate, yellowish green chlamidya trachomatis

- obligate intracellular, we cant culture it!! - azithromycin and doxycycline 2 serotypes: 1) serotypes D-K: NGU - ascending infections in both sexes. - exudate=> also purulent but more whitish and clear than gonorrhea 2) serotypes L1-L3 : lymphogranuloma venerum - 3 stages: a. tiny papules, flat ulcer b. painful lymphadenopathy=> bubos, abscess, fistula c. lymph obstruction due to strictures and fibrosis —> lymphedema, elephentiasis other NGU:

- mycoplasma uretritis- ureaplasma urealiticum- Serous discharge, sterile leukocyturia, dysuria

- trichomonas uretritis- trichomonas vaginalis- foul smelling yellow-green discharge, strawberry vagina

- mycoplasma vaginalis

hemophilus ducreI - chancroid: extremely painful genital ulcer

KEY WORDS 23) Lichen planus, pityriasis rosea, 1) lichen planus - An inflammatory, pruritic disease of the skin and mucous erythroderma membranes, and sometimes the nails which can be either generalized or localized - lesion: pruritic, shiny flat, purple papules, often with Wickman striae (white lines on the surface), the lesions may be discrete or coalesce to form plaques. - etiology: unknown, associated with HCV annular LP: - hitsto: inflammation of the dermo-epidermal junction with typical “saw-tooth” pattern with an intense upper dermal inflammatory infiltrate composed predominantly of T-cells - predilection: wrists, elbows, oral mucosa - variants of LP: 1) annular (penis mainly) 2) linear 3) follicular —> causes scarring alopecia! 4) pigmentosus 5) atrophic and hypertrophic 6) planopillaris

2) pytriasis rosea:

- Acute benign self- limiting scaly papular skin eruption of unknown etiology.

- lesion: Herald patch, salmon-coloured papules and plaques with fine collarette scale lesions.

- distribution: along the long axis is parallel to the lines of cleavage (“CHRISTMAS TREE”), generalized, affecting chiefly the trunk mucousal LP

3) erythroderma: “red men syndrome”

- not a disease by itself, clinical sign - lesion: marked erythema, red skin, scaly, desquamation - common etiologies: 1) extensionn of preexisting skin disorder—> most often adverse drug reaction 2) idiopathic- 30% examples: Sezary syndrome!!, atopic derm, psoriasis erythrodema, drug eruptions - treatment: underlying disease, discon. medication Lichen planopillaris

KEY WORDS 24) Erysipelas. Cellulitis. Necrotizing soft tissue infections (incl. fasciitis necrotisans)

erysipelas:

1) erysipelas: - STREPTOCOCCUS - superficial form of cellulitis—> upper dermis, superficial lymphatics, breach in the skin barrier, risk factors (venous disease, injury, diabetes), clinical features (well-defined border!!,pain, warm, swollen!!), legs+FACE - Characteristics include pink-to-red lesions that spread rapidly and are warm to the touch - severe forms: Bullous, hemorrhagic, gangrenous, accompanied by abscess formation, migrating, recurrent.

2) cellulitis: non-contagious!

- most commonly STREPTOCOCCUS+1/3 stap. - deeper dermis + subcut. fat, limbs, trauma/surgical wounds, fever, warm!!, pain, vesicles, abscess, lymphangitis!!),

- septicemia complication!

* how to differentiate: Being more superficial, erysipelas presents with a raised, distinct border. In contrast, the advancing border of cellulitis tends to be rather indistinct. In practice it is difficult to tell how deep the skin involvement is, and therefore to differentiate between cellulitis and erysipelas. It is probably best to consider erysipelas as a cellulitis with superficial spread. 3) necrotizing fascitis:

- 3 types:

1) type 1 (polymicrobial- aerobic+anaerobic) 2) type 2 (strep. pyogenes, “flash-eating” disease) 3) type 3 (gas gangrene) - soft tissue and fascia covering the muscles, life threat (75% mortality) - lesion: Intense pain, edema, livid discoloration rapidly developing

KEY WORDS 25) Erythema multiforme. StevenJohnsons syndrome. Lyell’s syndrome

Erythema multiforme: - HSR reaction, possibly mediated by deposition of immune complex (mostly IgM) in the superficial microvasculature of the skin and oral mucous membrane - etiology: 1. infection-90% (HSV-1, mycoplasma) 2. drug- 10% (penicillins, phenytoin, barbiturates) 3. idiopathic - distribution: acral (back of the hand), face, palms and soles, face, penis (starts at the back of the hands —> spread to trunk) - macule—> papule —> vesicles and bullae, target lesionsclassic and non classic, polymorphous eruptions at different stages, Kobner phenomenon - EM minor (no mucous membrane involve, classic target, vesicles no bullae, no prodrome/systemic illness) vs. EM major ( mucousa involved, bullae and Nikolsky sign, prodrome) SJS/Lyell syndrome:

- lesions: macules, targets, blisters, erythema, blisters unite -

together to for skin detachment, +Nikolsky, prominent mucous involve. variants of the same condition; classification by skin detachment: in SJS 3-10% overlap: 11-30% Lyell: >30% nearly always medications: antibiotics (sulfonamides, penicillin), allopurinol, anticonvulsants (lamotrigine, phenytoin, carbamezapine), NSAIDS (ibuprofen) prodrome!!!! (fever, cough, sore throat, runny nose) —> abrupt onset of painful skin rash extending rapidly hitsotolgy: keratinocyte/full thickness epidermal necrosis, separation of epidermis from dermis etiology: unknown, but highly associated with CD8+ T cells

KEY WORDS 26) Skin symptoms of DM and IBD (incl. erythema nodosum, pyoderma gangrenosum)

“Shin spots” in diabetic dermopathy

1) DM: diabetic dermopathy (“shin spots”= light brown scaly patches on the shins), acanthosis nigricans, diabetic bullae, lipodermatosclerosis, diabetic foot, pigmentation, xanthom/ xanthelasma, necrobiosis lipoidica, dermatitis, pruritus, prurigo nodularis, aurantiasis, diabetic cheiroartropathy (diabetic stiff skin), atrophie blanche other: superficial thrombophlebitis, erysiphelas/cellulitis, granuloma annulare necrobiosis lipoidica: one or more tender yellowish brown patches develop slowly on the lower legs over several months. They may persist for years. They may be round, oval or an irregular shape. The centre of the patch becomes shiny, pale, thinned, telangiectasia. A minor injury to an established patch can cause it to ulcerate

necrobiosis lipoidica:

2) IBD: Crohn's: erythema nodosum, perianal fissures and abscesses, granulomatous chelitis (lip granuloma), aphthous ulceration, pyoderma gangrenous, skin tags UC: erythema nodosum, pyoderma gangrenous

3) erythema nodosum:

- Deep, firm and tender reddish-blue painful nodules, never ulcerate

pyoderma gangrenosum:

- panniculitis, erythematous nodules, shines, circulating immune complexes deposited in bv of subcut. fat,

- etiologies (idiopathic=30%, bacterial, malignancy, IBD, viral) 4) pyoderma gangrenosum: no infection! no gangrene!

- Pyoderma gangrenosum is a rare neutrophilic vasculopathy

erythema nodosum:

that is often associated with chronic inflammatory diseases (IBD, RA). Autoimmune inflammatory neutrophilic dermatoses - pustule —> painful ulcer - start suddenly, often at the site of minor injury. - One hallmark of pyoderma gangrenosum is pathergy, which is the appearance of new lesions at sites of trauma. (Pathergy is a skin condition in which a minor trauma such as a bump or bruise leads to the development of skin lesions or ulcers that may be resistant to healing. Pathergy can also lead to ulcerations at the site of surgical incisions. Pathergy seen with both Behçet's disease and pyoderma gangrenosum.[1] A highly similar phenomenon known as Koebner occurs in autoimmune diseases such as psoriasis and systemic lupus erythematosus, among others.)

KEY WORDS 27) Mycosis fungoides, Sézary syndr., Kaposi’s sarcoma

- explain what is cutaneous T cell lymphoma (a type of an extranodal lymphoma: when it occur only in the skin—> primary skin lymphomas —> can be cut. T cell lymphomas- 65% (and cut. B cell lymphoma- less common)—> 2 types: indolent type (mycosis fungicides + its variants) and aggressive type (sezary syndrome)

MF:


 Mycosis fungoides:

- Neoplastic proliferation of mature CD4 T cells that infiltrate the skin, producing localized skin rash, plaques, and nodules.

- Aggregates of neoplastic cells in the epidermis are called Pautrier microabscesses.

- Cells can spread to involve the blood, producing Sezary sezary syndrome:

syndrome.

- 3 stages:

1) patch stage 2) plaque stage 3) tumor stage - variants: follicular MF, pagetoid reticulosis, granulomatous slack skin - PUVA, UVB311, topical steroids, oral retinoids, chemo (only if lymph involve) sezary syndrome: aggressive CTCL

- when mycoses fungicides spread to the blood - Characteristic lymphocytes with cerebriform nuclei (Sezary cells) are seen on blood smear

- generalized exfoliative erythroderma, intensive pruritus -

lichenificatiom, baldness and diffuse alopecia, nail dystrophy, palms-plantar hyperkeratosis treatment: like MF, but also ECP (blood exctracted—> irradiated —> turn back)

kaposi sarcoma:

- multicentric malignant neoplastic proliferation of the vascular endothelium.

- It’s associated with infection with the HHV-8 (Kaposi’s associated herpesvirus) and immunosuppression. The cell of origin is most likely the lymphatic endothelial cell. - lesion: bluish-red cutaneous nodules, usually on the lower extremities, most often on the toes or feet, and slowly increasing in size and number and spreading to more proximal areas - 4 types: 1) classic type (Jewish and Italian males in Europe and the United States) 2) HIV-associated (pandemic) 3) African (endemic) 4) Iatrogenic (due to drug treatment causing immune suppression) - treatment: radiation + chemo+cryotherapy with liquid nitrogen

KEY WORDS 28) cutaneous vasculitis polyarteritis nodosa:

most common symptoms: petechia, purpura, palpable purpura ecchymosis papules urticaria necrosis, ulcer nodules livedo reticularis vesicle, pustules

-

- Vasculitis is an inflammatory condition, which damages the

HSP:

Kawasaki

blood vessels. It may be primary or associated with underlying disorder (autoimmune disease, drug reaction, infection, malignancy, serum sickness). - These diseases are classified according to the Chapel Hill classification, which is based on the size of the vessel involved: 1) large vessel vas.: giant cell arteritis 2) medium vessel vas.: poly nodosa, kawasaki 3) small vessel vas.:microscopic polyangitis, WG, Churg Straus, HSP 4) variable vessel vas.: Bechet disease 5) single organ vas.: cutaneous PAN 6) vasculitis associated with systemic disease: Lupus vasculitis 7) other: tumor associated vas., hypersensitivity vasculitis (e.g., due to drugs/infection)

Bechet disease:

- immune mediated small/medium vessel vasculitis manifest as mucosal lesions of the oral mucosa, eyes, genital ulcers

livedo reticularis:

-

KEY WORDS 29) Photodermatoses (incl. porphyria cutanea tarda, photoallergic and phototoxic skin reactions, PLE)

- definition of photodermatosis (Photodermatosis is a skin disease

PLE:

- diagnosis procedures: clinically, photos (MED/MUD, provocation

that is caused by exposure to sunlight…).

- talk about UV radiation (UVA, UVB) in terms of: wave length, skin penetration, skin effect

- etiology of photodermatoses: UV with or without photosensitizer, hormones/metabolic processes etc.)

test, photo patch test), lab tests (porphyrin in RBC/urine/serum)

- Photopatch tests are additional patch tests applied when the

solar urticaria:

dermatologist suspects contact allergy to a substance, occurring only in the presence of sunlight. After the additional patches are removed, they are exposed to a small dose of long wave ultraviolet light (UVA). - classification: 5 types: 1) idiopathic photodermatoses: PLE, Juvenile spring eruption of the ears, Solar urticarias (SU) 2) photosensitivity secondary to external agents: phytotoxic dermatitis, Photoallergic contact dermatitis 3) cutaneous porphyries: EPP, hepatic: PCT 4) geno-photodematoses: xeroderma pigmentosum 5) photoagravated disorders: SLE, albinism, pemphigus, rosacea PLE:

- rash after sunlight exposure: Acneiform, urticarial, erythema multiforme-like or blistering lesions

- diagnosis: provocation phototest

Juvenile spring eruption of the ears:

- papules and vesicles on the ears

Solar urticaria: (type of physical urticaria)

PCT

- itching, immediate wheals due to sun exposure - Diagnosed with MUD and provocation test Phototoxic dermatitis:

- when certain chemicals are applied to the skin and subsequently exposed to the sun causing dermatitis

- provoking: psoralen, cosmetics. drugs: tetracyclins - erythema, edema, blisters. Heals with long-lasting

EPP

pigmentation Photoellergic contact dermatitis: - a papulovesicular, eczematous, or exudative skin reaction that occurs 24 to 48 hours after exposure to light in a previously sensitized person. - example: taking sulfonamide drug —> it concentrates in the skin and requires chemical alteration by light to become an active antigen —> second exposure to sunlight —> allergic response - Diagnosis via photopatch test, “crescendo” type reaction Cutaneous porphyria: - EPP (erythropietic protoporphyria): inherited AD, Enzymatic defect of ferrochelatase, scarring, waxy thickening, orange peel nose - PCT (porphyria cutanea tarda): most common porphyria, Uroporphyrinogen decarboxylase defect, Fragile skin that blisters, erosions, scars and milia, Hypertrichosis, pigmentation Xeroderma pigmentosum: - Autosomal recessive, due to deficiency in the enzyme that permits excisional repair of ultraviolet-damaged DNA. Pigmented spots, early malignancies, Fatal outcome

KEY WORDS 30) Hair disease (separate)

-

hair growth cycle (anagen —> catagen —> telogen) hair types (lanugo, villus, terminal) hair diseases: hair loss and hair gain hair loss: 1. non-scarring alopecia: - pattern hair loss (males vs. women, Hamilton vs. Ludwig classification, minoxidil, finasteride) - alopecia areata: AA, AAU, AAT, ophiasis (autoiimune T cells against hair follicle) - telogen effluvium: hormonal. nutritional, drugs, stress - anlagen affluvium: radiation and chemotherapy!! 2. scarring alopecia: CCLE, Lichen planopillaris, “kerion” and favus tinea capitis, surgical scar, neoplasm hair growth: - hypertrichosifs —> in a non-androgen dependent pattern, lanugo/terminal hair (drugs, hormones, congenital, porphyria - hirsutismm —> in a androgen dependent pattern, villus hair (drugs, tumor secreting androgens, idiopathic)

KEY WORDS 31) Autoimmune bullous disorders (incl. pemphigus, pemphigoid, dermatitis herpetiformis)

Bullous diseases are defined as conditions where cavities filled with fluid form in the superficial layer of the skin clinically manifesting as vesicles or blisters. Although vesicle and blisters can arise as secondary lesions in many conditions, in the bullous diseases they are the primary pathologic event. Genetic (hereditary) and acquired (mostly autoimmune) bullous diseases exist

Pemphigus: 2 major types: PV, Pemphigus foliaceous (PF) 1) Pemphigus foliaceous (PF): - no mucous lesions - starts with scaly crusted lesions on an erythematous base, initially on seborrheic areas (back). 2) pemphigus vulgaris (PV): - Autoimmune destruction of desmosomes between keratinocytes - Due to IgG antibody against desmoglein (type II HSR) - Presents as skin and oral mucosa bullae. - histology: Acantholysis (separation) of stratum spinosum keratinocytes (normally connected by desmosomes) results in suprabasal blisters. Basal layer cells remain attached to basement membrane via hemidesmosomes. - Thin-walled bullae rupture easily (Nikolsky sign), leading to shallow erosions with dried crust. - Immunofluorescence highlights IgG surrounding keratinocytes in (a 'fish net' pattern). - starts in the oral mucosa (erosions, no bullae), later skin lesion bullous pemphigoid: .

pemphigus vulgaris

- Autoimmune destruction of hemidesmosomes between basal -

cells and the underlying basement membrane Due to IgG antibody against basement membrane collagen Presents as blisters of the skin; Basal cell layer is detached from the basement membrane Tense bullae do not rupture easily; clinically milder than pemphigus vulgaris, mucous membrane involvement —> only mouth, and less severe than PV. Immunofluorescence highlights IgG along basement membrane (linear pattern).


 dermatitis herpetiformis:

bullous pemphigoid:

- Autoimmune deposition of IgA at the tips of dermal papillae - Presents as pruritic vesicles and bullae that are grouped - Strong association with celiac disease; resolves with glutenfree diet

- patho.: IgA Ab to gliadin component of the gluten cross react with epidermal transglutaminase eTG —> IgA-eTG immune complexes despited in the papillary dermis

KEY WORDS 32) HPV- and Pox-virus induced skin infections. Viral exanthems

papilloma viruses family: HPV

- cutaneous or STD infections - hyperkeratosis leads to formation of wart - E6 and E7 proteins inhibit tumor supressor gene P53 cutaneous diseases: 1. verruca vulgaris (common wart): serotypes 1, 2 and 4, hands and fingers mainly 2. verruca plantaris (plantar wart): serotype 1 mainly 3. verruca plana (flat warts): serotypes 3, 10 STD: - oral mucosa and anogenital region - serotypes: 6, 11 most commonly. - serotypes 16, 18, 31, 33 are strongly associated with anogenital displasya and carcinoma - 4 clinical types of genital warts occur: condylomata acuminata, small papular, keratotic warts, flat topped papules congenital: - respiratory papillomatosis Pox viruses family: smallpox (variolla), Molluscum contagiosum, parapoxosvirus 1) variolla: smallpox, human only - variolla major (90%) and variolla minor (30%), eradicated due to vaccines, only in humans, respiratory system —> mucous membrane —> lymph nodes—> invade capillaries endotheliumm of the skin —> maculopapular rash —> vesicles—> pustules —> umbilication and crusting 2) MCV virus: Molloscum contagiousum, human only papules/nodules/tumor with central umbilication, discrete, solid. self limited. 3) paprapoxvirus: zoonosis Milker’s nodule, single/multiple eroded nodules occur at the site of inoculation

viruses induced exanthem:

- definition: exanthem is the medical name given to a widespread rash that is usually accompanied by systemic symptoms such as fever, malaise and headache. - Erythematous macules, papules, perhaps vesicles, petechiae - Usually on head, neck, trunk, proximal extremities - prodrome precedes skin symptoms 1) measles/rubeola (measles virus) - Kopliks spots=bluish-white papules in the oral mucousa 2 days before the rash, than maculopapular erythematous rash from head—> to trunk 2) rubella/german measles (rubella virus) - risk of congenital rubella, MMR 3) erythema infectiosum/slapped cheek (B19 virus) maculopapular rash of central distribution 4) roseola infantum /exanthema subitum (HHV-6 virus)- after fever fades, rash paper, enanthem and exanthema 5) chickenpox (varicella zoster virus)

KEY WORDS 33) CDLE, SCLE, SLE, Dermatomyositis. Progressive systemic scleroris. Morphea

dermatomyositis:

- autoimmune disease (mediated by CD8+ T-cells) that primarily affects the skin and striated muscle.

- In adults, it is a potential paraneoplastic marker for an underlying malignancy.

- clinical features: heliotrope rash (reddish-purple retythema on upper lids and deem on on lower lids), Gottron’s papules (MCP, interphalangeal joints), poikiloderma, seborrheic eczema, calcification in subcutaneous fascial tissue and calcinosis, alopecia, periungual telangiectasia

- 5 diagnostic criteria: 1. 2. 3. 4. 5.

symmetric proximal extremity muscle weakness positive result of muscle biopsy raised level of CK and LDH myopathy in EMG characteristic skin symptoms

systemic sclerosis (scleroderma):

- Progressive systemic sclerosis affects the skin and many -

-

internal organs. It is usually divided into acral or limited form and diffuse form. Major feature is the deposition of increased amounts of EC matrix molecules (mainly collagen) features: Masked or sclerotic faces with difficulty with wrinkling of forehead, Difficulty whistling, pointed nose, Microstomia, perioral radial folds, narrow lips, Telangiectasia of the nail bed & claw-like flexion of fingers, Calcinosis, scarring inflammation and fingertip necrosis, Shortening of digits and hyperkeratosis Raynauds phenomenon, Cataracts treatment: immunusupressio, vasoactive, UVA1 CREST syndrome is a variant: Calcinosis cutis Raynaud phenomenon Esophageal dysfunction/dysmotillity Sclerodactyly Telangiectasia

morphed (localized scleroderma): Red-violet macule or patch that spreads w. central sclerosis and peripheral lilac ring (ivory-colored, shiny plaque, lilac-colored, ill defined border)

KEY WORDS 34) Pathogenesis of AIDS, skin symptoms of HIV

- talk about the virus and its pathogenesis (HIV, RNA virus, retro

35) Burns, burn disease, Congelatio

- pathophysiology (protein damage, sensation, fluid leakage,

virus, reverse transcriptase enzyme, CD4+, macrophage, dendritic cells, low levels of CD4 through different mechanisms, lost of CMI at some level point and AIDS) - transmission - course: primary phase —> asymptomatic phase —> generalized lymphadenopathy phase —> symptomatic phase AIDS (high viral load, low CD4+count) - explain the graph - HAART treatment - clinical manifestations: 1. highly associated dermatologic disorders with HIV disease (acute retroviral syndrome, kaposi, eosinophilic folliculitis, pruritic papular eruptions, chronic herpes, leukoplakia) 2. moderately associated (molloscum contagiosum, candidiasis) 3. possible risk for HIV (generalized lymphadenopathy, seborrheic dermatitis, recurrent apthous ulcer) - adverse cutaneous drug effects in HIV disease (mainly due to sulfa drugs) —> exanthamtous/morbiliform eruptions, lipodystrophy (hyper+hypotrophy) inflammation risk)

- types of burns: - Thermal burns- These burns are due to heat sources which

-

raise the temperature of the skin and tissues and cause tissue cell death or charring. Hot metals, scalding liquids, steam, and flames, when coming into contact with the skin, can cause thermal burns. Radiation burns- These burns are due to prolonged exposure to ultraviolet rays of the sun, or to other sources of radiation such as X-ray. Chemical burns- These burns are due to strong acids, alkalies, detergents, or solvents coming into contact with the skin or eyes. Electrical burns- These burns are from electrical current, either alternating current (AC) or direct current (DC).

- 3 tables (comparative, Wallace rule of 9, severity of burns) - treatment (dressing, cooling, pain relief, burn-unit, skin grafts, amputations) congelatio: freezing temp, less than 0 degrees, vasoconstriction, extremities (toes, feet, fingers, ears, nose and cheeks), loss of sensation, necrosis, don't rewarm at the field chilblains (‫)אבעבועות קור‬: Chilblains are itchy and/or tender red or purple bumps that occur as a reaction to cold. Chilblains are also known as pernio or perniosis. They are a localised form of vasculitis.

KEY WORDS 36) Benign skin tumors (incl. tumors of the epidermis, fat and connective tissue, cysts and vascular nevi) Becker nevus:

miscellaneous benign neoplasm and hyperplasias: 1) seborrheicc keratosis: “greasy” feel and “stuck on” appearance, pigmented, round, flat, coin-like plaques, activating mutations of the fibroblast growth factor (FGF) receptor 3, eruptive/paraneoplastic form —> Sign of LesserTrélat 2) Becker nevus- pigmented hamartoma (i.e., developmental anomaly showing changes in pigmentation, hair growth and slightly elevate verrucous surface) 3) cylindroma 4) warts 5) trichoepithelioma - face, small sharply defined smooth papule 6) syringoma- benign adenomas of the eccrine ducts 7) epidermal nevus- developmental hamartoma showing hiperplasia of epidermal structures (epidermis+adnexa)

cylindroma benign dermal and subcutaneous neoplasms and hyperplasia:

- skin tags- more common in overweight people, face, neck, axilla

- dermatofibroma - lipomas- most common human tumor

trichoepithelioma (hair follicle tumor)

miscellaneous cysts and pseudocysts: *closed spaces surrounded by an epithelium, contain fluid or cellular secretions - epidermoid cyst- most common cyst, surgical excision - Milium

Hypertrophic scars and keloids - keloids (extends beyond the boundaries)

vascular tumors and malformations: 1. tumors: - hemangiomas: hemangioma of infancy, congenital syringoma: (sweat gland tumor)

2. malformations: spider angioma venous lake cherry angioma telangiectasia port-wine stain (neavus flammeus): almost always a birthmark, grows with the patient, nodules may appear later, may be part of a syndrome such as Sturge–Weber syndrome or Klippel– Trénaunay–Weber syndrome, laser treatment

-

epidermal nevus

KEY WORDS 37) pigment cell nevi. vitiligo

Naevi may form from other skin cells (e.g. vascular naevi are formed from blood vessels), but only those derived from melanocytes are known as moles.

pigment cell nevi: common skin lesion, due to a proliferation of the pigment cells, melanocytes. If they are brown or black in colour, they may also be called melanocytic naevi. classification: 1) Epidermal: Cafe-au-lait, melanocytic neavus, naevus spilus, lentigo simplex 2) Dermal: Mongolian spot, blue nevus 3) Epidermal and dermal: congenital melanocytic nevi 4) Congenital: Small: < 1,5 cm, Medium: 1,5-20 cm, Giant: > 20 5) Acquired * talk about: 1) congenital nevi: small/medium/giant congenital NMN, cafe-aulait, Mongolian spot, nevus of Ota 2) acquired nevi: common nevomelanocytic nevus, Spitz nevi, Halo nevus, Blue nevi, nevus spilus, dysplastic nevus *nevomelanocytic nevus (NMN):

- benign neoplasm of melanocytes. - congenital and acquired (congenital NMN may be precursor for malignant melanoma!!)

- characterized by flat macule or raised papule with symmetry, sharp borders, evenly distributed color and small (<6mm) 3 types: 1) junctional (intarepidermal) —> flat 2) compound (both) —> slightly elevated/dome-shaped 3) dermal (intradermal) —> elevated - dysplasia may arise which is a precursor for melanoma

Familial dysplastic nevus syndrome or FAMM: Atypical naevi that run in families may be part of the FAMM syndrome. FAMM is an abbreviation for Familial Atypical Mole and Melanoma. People with FAMM syndrome must have the following: ▪ One or more first-degree or second-degree relative with malignant melanoma; ▪ A large number of naevi (often more than 50), some of which are atypical naevi; ▪ Naevi that are dysplastic on histopathology.

dysplastic nevus: precursor of cutaneous melanoma de novo or from existing melanocytic nevus (usually compound) clinically distinctive from common acquired nevi; ABCDE role potential precursor of superficial spreading melanoma (SSM) - can occur either sporadically or in the familial forms—> familial dysplastic nevus syndrome (AD inheritance, risk of melanoma approaches 100%) - “out of step” appearance —> a mix of large and small, flat and raised, tan and very dark lesions (in contrast to nevomalanocytic nevi which are usually in a roughly comparable stage of development in a given body region, i.e. junctional, compound, dermal) - morphology: somewhere between NMN and SSM

-

Vitiligo:

- genetic background exist - distribution: focal type and generalized type, SYMMETRIC!! - predilection sites: around eyes and mouth, digits, elbows, knees, low back, “lip-tip" pattern

- pathogenesis: 3 theories: 1) autoimmune theory —> melanocytes destruction by lymphocytes 2) neurogenic hypothesis —> interaction of meloncytes with nerve cells 3) self destruct hypothesis—> meloncytes destruction by toxic substances - treatment: sunscreens, oral-PUVA with 5-MOP, UVB311

KEY WORDS 38) Malignant melanoma

classification: 1) De novo melanoma: a. melanoma in situ b. lentigo maligna melanoma c. superficial spreading melanoma d. noduler melnoma e. acral lentiginous melanoma f. melanoma of the mucous membranes g. desmoplastic melanoma 2) melanoma arising from precursors: a. from dysplastic NMN b. from congenital NMN c. from common NMN

- 30% —> from preexisting melanocytic lesion, 70% —> in normal skin

- early recognition and excision —> of all cancers melanoma is the most rewarding for detection of early, curable primary tumors

- etiology and pathogenesis: unknown. role of genetic lentigo maligna: - least common - sun exposed - sunlight=most important pathogenic factor - old patient - start as lentigo maligna=MIS superficial spreading:

- most common - moderately slow growing nodular:

- second most common - middle life persons with white skin - on less commonly exposed sun areas acral lentiginous:

- mostly in Asians, Africans - older males (>60) - poor prognosis due to the delay in the development (the tumor is discovered very late)

predisposition and sun exposure. Melanoma prone-families shows CDKN2A mutation in 25%. other mutations- in tumor suppression genes: BRAF (66%) - risk factors: CDKN2a, BRAF, skin type 1-2, family history of dysplastic nevus or melanoma, UV, more than 50 nevi and more than 5mm, congenital nevi - sun exposure —> solar radiation is the major cause of cutaneous melanoma - growth patterns: radial growth phase —> vertical growth phase - evaluations: Breslow thickness (stages 1 through 5; less than 0.75mm to more than 3mm in stage 5) and Clarcks level (levels 1 through 5 starting from epidermis till the invasion to the subcutaneous tissue) - 4 major types of melanoma: 1) superficial spreading- any site, delayed vertical 2) lentigo maligna - mainly sun exposed, radial for years 3) nodular- any site, immediate vertical 4) acral lentiginous - palms and soles, radial for months to years *explain them in terms of: site, radial and vertical growth - superficial spreading—> most common (70%)! - lentigo maligna melanoma—> least common (5%)!

- ABCDE of melanoma A- asymmetry in shape B- irregular borders C- color is not uniform D- diameter is large E- Elevated, irregular surface, Evolving- history of increase in the size - morphologies: 1) LMM—> typical variation in color and highly irregular in shape 2) SSM—> elevated, flat lesion (plaque), striking pigment variation 3) nodular—> uniformly elevated, thick plaque or an exophytic. polypoid, or dome-shaped lesion

KEY WORDS 40) Precancerous tumors, in situ malig. , SCC & BCC

- risk factors:

epidermal precancerous lesions:

palmoplantar keratosis:

1) most common etiology —> UV radiation, HPV 2) less common —> chronic inflammation, ionizing radiation, hydrocarbons, industrial carcinogens, inorganic arsenic UVR and HPV have the following spectrum of changes: epithelial dysplasia —> SCCIS —> invasive SCC

Bowen disease

differentiated SCC

classification of epithelial precancerous lesions and SCCIS: 1) UV-induced: solar keratosis, lichenoid actinic keratosis, Bowen disease (SCCIS) 2) HPV- induced: low-grade squamous-intraepithelial lesion (HSIL), Bowenoid papulosis (SCCIS) 3) arsenic-induced: palmoplantar keratosees, Bowenoid arsenical 4) keratosis and hydrocarbon keratoses 5) thermal keratoses *Actinic (solar) keratosis: Morphology: Good way to remember the histologic features is SPAIN: S: Solar elastosis P: Parakeratosis A: Atypia (keratinocytic) I: Inflammation (lymphocytes in the superficial dermis) N: Not full thickness (atypia) *SCCIS:

- Bowen disease- red sharply defined scaly plaque on the skin, Erythroplasia- similarly but non-scaly, on the genitals

- induced by HPV (anogenital region)—> Bowenoid papulosis invasive SCC:

- arises in epidermal precancerous lesion (see above) - 2 types can be distinguished: non-differentiated SCC:

keratoacanthoma

1) Differentiated SCC 2) Non-differentiated SCC THUMB RULE: —> differentiated SCC is hard on palpation and has hyperkeratosis —> undifferentiated SCC is soft and has NO hyperkeratosis • lesions: - differentiated SCC: indurated papule, plaque or nodule, with adherent thick keratotic scale/hyperkeratosis - non-differentiated SCC: fleshy, granulating, easily vulnerable arose papule and nodules. ulceration, bleeds easily • locations: - differentiated SCC: sun-exposed, scalp, lower lip, cheek - non-differentiated SCC: genitalia (when arise from erythroplasia), extremities/trunk/face (when arise from Bowen disease) keratoacanthoma: a variant of SCC rapidly growing, may regress spontaneously/metastasize HPV 9, 16, 19 have been identified in KA lesion: dome shape with central keratotic plug

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KEY WORDS BCC Superficial multicentric

- most common tumor - does not metastasize (depends on growth factors from the surrounding storm for its growth)

- etiology: UVR (especially UVB), PTCH mutation - locally invasive, aggressive and destructive but slow growing

- 5 different types:

nodular BCC

pigmented BCC

1) superficial multicentric BCC: - thin plaque, pink or red, characteristic threadlike border and telangiectasia - the only type that can exhibit scales - may evolve to nodular or ulcerating BCC 2) Nodular BCC: - papule or nodule, translucent- “pearl-like” nodule, skin-colored or reddish, telangiectasia 3) Ulcerating BCC: - ulcer with a rolled border (rodent ulcer), translucent, telangiectasia, firm 4) Sclerosing BCC: - look like small morphea patch or scar, ill-defined, whitish color - excessive amount of fibrous stroma and a finger-like growth histology —> must remove it with wide margins - may evolve to nodular or ulcerating 5) Pigmented BCC: - smooth, glisening, hard and firm - may be indistinguishable from melanoma

distribution:

- >90% on the face - superficial BCC - on the trunk - no mucosal involvement as this cancer grows only where there is a hair follicle Treatment:

sclerosing BCC

- Mohs surgery - cryosurgery, electrosurgery - 5-FU, imiquomod