Mucocutaneous Symptom Complex Part 2

AN OVERVIEW OF MUCOCUTANEOUS SYMPTOM

COMPLEX Part 2

ANTONIO E. CHAN, M.D.

Classification

• Maculopapular eruption • Vesiculobullous or vesiculopustular • Petechial or purpuric eruption

Definition & Pathogenesis Blisters are circumscribed elevated lesions filled with clear fluid – Vesicles measure 5 mm or less in diamter – Bullae measure more than 5 mm in diameter

Definition & Pathogenesis

• Result from a disturbance of cohesion of epidermal cells or components of the basement membrane zone associated with influx of fluid into or beneath the site of disturbance

Definition & Pathogenesis • Depending on the mechanisms responsible for their formation, blisters can contain a combination of edematous or lymphatic fluid, serum proteins, antigen-antibody complexes, and soluble inflammatory mediators.

Pertinent Questions To Ask • Patient’s age • Season • Exposure to infectious agents or medications • History of previous disease • Concurrent signs & symptoms • Morphology, distribution and evolution of the rash

Viral Etiology of Vesiculobullous Lesions

• Varicella-zoster (wild and vaccine strains) • Herpes simplex virus type 1 & 2 • Coxsackievirus A4, A5, A7-10, A16 & B1-3, B5 • Echovirus 4, 6, 9, 11, 17, 19, 33 • Enterovirus 7, 2 • Molluscum contagiosum

Bacterial Etiology of Vesiculobullous Lesions

• Staphylococcus aureus – Bullous impetigo – Scalded skin syndrome • Ritter’s disease • Lyell disease

• Streptococcus pyogenes – – – – –

Blistering distal dactylitis Ecthyma Erysipelas Non-bullous impetigo Scalded skin syndrome

• Disseminated gonococcal infection

Non-infectious Etiology of Vesiculobullous Lesions

• Stevens-Johnson syndrome • Thermal injury • Arthropod bites (Cimex spp.[bedbug], Sarcoptes scabies) • Contact dermatitis

VARICELLA

VARICELLA

“dew drop-like lesions”

Skin lesions at various stages of evolution

HERPES ZOSTER (SHINGLES)

ACUTE HERPETIC GINGIVOSTOMATITIS

Herpetic whitlow

MOLLUSCUM CONTAGIOSUM (WART)

Umbilicated lesions

IMPETIGO CONTAGIOSA

STAPHYLOCOCCAL SCALDED SKIN SYNDROME Sunburst radial pattern

Positive Nikolsky sign

DISSEMINATED GONOCOCCAL INFECTION (ARTHRITIS-DERMATITIS SYNDROME)

 Discrete tender necrotic pustules with erythematous base  Arthritis

ERYTHEMA MULTIFORME

(STEVENS-JOHNSON SYNDROME)

Cutaneous lesions are symmetric, in crops and show predilection for the extensor surfaces of the hands, arms, feet, legs, palms and soles

ERYTHEMA MULTIFORME

(STEVENS-JOHNSON SYNDROME)

Viral Etiology of Petechial or Purpuric Lesions

• Dengue virus (DHF) • Measles virus (Black measles) • Rubella virus (Congenital rubella syndrome) • Enterovirus

Bacterial Etiology of Petechial or Purpuric Lesions

• Neisseria meningitidis (Septicemia)

Non-infectious Cause • Henoch Scholein Purpura

Aedes aegypti  Dengue transmitted by infected female mosquito  Primarily a daytime feeder  Lives around human habitation  Lays eggs and produces larvae preferentially in artificial containers with clean stagnant water

Dengue Clinical Presentations  Undifferentiated fever – may be the most common presentation*  Classic dengue fever  Dengue hemorrhagic fever (DHF)  Dengue shock syndrome (DSS)

Clinical Characteristics of Dengue Fever



In infants and young children –

Undifferentiated febrile illness

–

Maculopapular rash

–

Petechiae and a positive tourniquet test, not uncommon

Clinical Characteristics of Dengue Fever 

Older children – Mild febrile illness, or – High fever with  Severe headache  Pain behind the eyes  Muscle and joint pains  Rash  Petechiae and a positive tourniquet test

Clinical Case Definition for Dengue Hemorrhagic Fever 4 Necessary Criteria  Fever, or recent history of acute fever  Hemorrhagic manifestations  Low platelet count (100,000/mm3 or less)  Objective evidence of “leaky capillaries:”  elevated hematocrit (20% or more over baseline)  low albumin  pleural or other effusions

Clinical Case Definition for Dengue Shock Syndrome  4 criteria for DHF  Evidence of circulatory failure manifested indirectly by all of the following:  Rapid and weak pulse  Narrow pulse pressure (≤20 mm Hg) OR hypotension for age  Cold, clammy skin and altered mental status  Frank shock is direct evidence of circulatory failure

Tourniquet Test • Inflate blood pressure cuff

to a point midway between systolic and diastolic pressure for 5 minutes

• Positive test: 20 or more petechiae per 1 inch2 (6.25 cm2)

Dengue Hemorrhagic Fever Grading the Severity Grade

Manifestations

I

Fever Non-specific constitutional symptoms such as anorexia, vomiting & abdominal pain

II

Manifestations of grade I plus Spontaneously bleeding

III

Manifestations of grade II plus Circulatory failure

IV

Manifestations of grade III plus Profound shock

Hemorrhagic Manifestations of Dengue

 Skin hemorrhages: petechiae, purpura, ecchymoses  Gum bleeding  Nose bleeding  Gastro-intestinal bleeding: hematemesis, melena, hematochezia  Hematuria  Increased menstrual flow

Dengue rash with blanching

DENGUE HEMORRHAGIC FEVER

Herman’s rash

Meningococcal Infection

• Serogroups A, B, C, D, H, I, K, L, S, Y, Z, W135 & 29E A, B, C account for more than 90% of meningococcal disease worldwide • The human nasopharynx is the only natural reservoir • Transmission: respiratory droplet and requires close and direct contact. • Asymptomatic carriers are the most common source of transmission

Meningococcal Infection • The spectrum of disease ranges from asymptomatic transient bacteremia which clears spontaneously to fulminant sepsis resulting in death only a few hours after the first symptoms occur • The incubation period of invasive disease is short

Meningococcal Infection • Risk factors associated with invasive disease Host factors – Young age – Crowding – Lower socio-economic class – Concurrent upper respiratory infection – Specific immune deficiencies (properdin or terminal complement) – Functional or anatomical asplenia – Smoking (active or passive)

Meningococcal Infection

• Risk factors associated with invasive disease Pathogen factors – Lipo-oligosaccharide (LOS) – pontent endotoxin – Hypervariability of surface antigen

MENINGOCOCCAL INFECTION

Meningococcal infection

Meningococcemia

WATERHOUSE FREDERICHSEN SYNDROME

Hemorrhagic Measles (Black Measles) • A rare but fatal occurrence • Characterized by sudden onset of high fever accompanied by seizure or altered mental state • Pneumonia, hemorrhagic exanthem & enathem • Bleeding from the mouth, nose, gastrointestinal tract & probable DIC

HENOCH SCHONLEIN PURPURA

• A vasculitis of small vessels • Etiology unknown • Typically follows URTI • 2 – 8 yr. of age • Skin lesions appear in crops • Rash begins as pinkish maculopapules initially blanch on pressure and progress to petechiae or palpable purpura (red – purple – rusty brown) • Predilection over the dependent areas or areas of greater tissue distensibility

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