Pedia: Mucocutaneous_symptom_complex

1 AN OVERVIEW OF MUCOCUTANEOUS SYMPTOM COMPLEX ANTONIO E. CHAN, M.D. (Module 4 Lecture date: June 29, 2006) DEFINITION A febrile illness in children associated with skin manifestation (exanthem) and mucous membrane involvement (conjunctiva, throat, respiratory or gastrointestinal tract) CLASSIFICATION • Maculopapular eruption • Vesiculobullous or vesiculopustular • Petechial or purpuric eruption

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OTHER CAUSES OF MACULOPAPULAR ERUPTIONS • Kawasaki disease • Drug eruption

ESSENTIAL ELEMENTS OF HISTORY (Pertinent questions to ask) •

MACULES are circumscribed, flat, discolored lesions that are not palpable and less than 1 cm in diameter PAPULES are circumscribed, solid, elevated lesions, less than 1 cm. in diameter

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DESCRIPTIVE DERMATOLOGIC TERMS Lesions Discrete (Rubelliform) Confluent (Morbilliform) Reticulated

Description Lesions remain separate

Example Childhood exanthems

Lesions run together

Childhood exanthems

Lace-like network

Erythema infectiosum

Multiform (Polymorphous) Iris

More than one type of shape or lesion Circle within a circle; A bull’s eye lesion Lesions clustered together Widespread Linear arrangement along a nerve distribution

Erythema multiforme

Grouped Generalized Zosteriform

Erythema multiforme

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Many different types of viruses, treponemes, chlamydia, rickettsiae, mycoplasma, bacteria, fungi, protozoan and metazoan agents cause illness with associated cutaneous manifestations Many possible etiologic agents; hence, no unified epidemiology exist. Erythematous macules and papules are the most common primary lesions seen during acute febrile illness in children Occurring in association with mild, febrile upper respiratory or gastrointestinal tract illness In the recent era, enteroviruses are the leading cause of infection-related exanthematous diseases Most exanthematous illnesses in children are benign, their differential diagnoses is critical because the early manifestations of potentially fatal bacterial and rickettsial diseases frequently have cutaneous findings. Many conditions that will ultimately manifest purpuric, vesicular, urticarial or ulcerative cutaneous lesions may first appear as erythematous macules or papules Maculopapular rashes are non-specific, a review of epidemiologic and physical findings is most helpful in establishing a diagnosis

Dissemination of infectious agents by blood (viremia, bactermia) which results in secondary infection at the cutaneous site a.

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• Hesrpes zoster

PATHOGENESIS 1.

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Herpes simplex

GENERAL STATEMENTS •

Streptococcus pyogenes – Scarlet fever Salmonella typhe – Typhoid fever Leptospira spp. - Leptospirosis N. meningitidis – Meningococcemia early N. gonorrhea – disseminated Bartonella henselae – Cat Scratch Disease Streptobacillus moniliformis – Rat bite fever

Direct result of infectious agents in the epidermis, dermis or dermal capillary endothelium b. An immune response between the organism and antibody or cellular factors in the cutaneous location. Dissemination of known specific toxins of infectious agents

3. A combination of these mechanisms VIRAL CAUSES OF MACULOPAPULAR ERUPTIONS • Rubeola – Typical, Modified, Atypical & Hemorrhagic Measles • Rubella virus - German measles • HHV 6 & 7 – Roseola infantum (Exanthem Subitum) • Parvovirus B19 – Erythema infectiosum • Enteroviral infection – Enterovirus 71 – Coxsackievirus – A2, A4, A5, A7, A9, A10, A16, B1B5 – Echovirus – 1-7, 11-14, 16-19, 22, 24, 25, 30, 38 • Epstein Barr Virus – Infectious mononucleosis • Hepatitis B virus – Papular Acrodermatitis in Childhood • HIV BACTERIAL CAUSES OF MACULOPAPULAR ERUPTIONS • Staphylococcus aureus – SSSS, TEN

Demographic data – Age – Season – Geographic area Exposure – Ill contacts – Sexual contacts – Travel – Pets, wildlife, insects (esp. ticks) – Medications and drugs – Transfusions – Immunizations Features of the rash – Temporal associations (onset of rash relative to fever) – Progression and evolution – Location and distribution – Pain or pruritus Associated signs & symptoms – Focal (suggesting organ specific illness) – Systemic (suggesting generalized or multi-system illness) History of previous illness (infectious)

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ACUTE FEBRILE ILLNESS WITH MACULOPAPULAR ERUPTIONS DISEASE OR SYNDROME RUBEOLA (Measles)

INCUBATION PERIOD (DAYS)

INFECTIOUS AGENT Infants, Adolescents Infants, young adults

RUBELLA (German Measles) ROSEOLA INFANTUM (Exanthem Subitum)

HHV-6 & 7

ENTEROVIRAL INFECTION

Coxsackieviruses A2, A4, A5, A7, A9, A10, A16, B1-B5; Echoviruses 1-7, 11-14, 16-19, 22, 24, 25, 30, 38; Enterovirus 71

Mild symptoms with onset 1-5 days before rash. Fever usually <38.50C. Headache, malaise, and suboccipital and postauricular lymphadenopathy

Erythematous, maculopapular, discrete

15 – 21

Fever 3-5 days in duration, rapid defervescence, and then the appearance of rash. No prodromal period Fever and mild to moderate pharyngitis. Occasionally, herpangina, meningitis, and other manifestations of systemic viral infection. Exanthem occurs in 5-50% of infections, depending on virus type. Rash may occur during fever or after defervescence. Hand, foot, and mouth syndrome

Erythematous, macular or maculopapular

Biphasic illness with mild prodromal period with headache and malise for 2-3 days, then 7-day symptom-free period, followed by typical exanthem

Starts on face. More prominent on extensor surfaces of extremities.

Fever, pharyngitis, myalgia, arthralgias, adenopathy, and rash

Three stage exanthem: Initially, rash on cheeks (slapped-cheek appearance) and then erythematous maculopapular rash on trunk and limbs. Finally rash develops a reticular pattern Most commonly erythematous, macular, maculopapular, and discrete (rubelliform). In association with ampicillin administration, the rash may be more vivid. Erythema multiforme and urticaria may occur Maculopapular, macular, and/or urticarial. In young children, papular (Gianotti-Crosti syndrome). Rarely, erythema multiforme Macular

Usually occurs in infants and children 1 month - 5 years of age. Mucopurulent nasal and eye discharge. Fever and staphylococcal infection in throat, but no evidence of pharyngitis

Scarlatiniform eruption with exfoliation. Positive Nikolsky sign. Crusty appearance around eyes and under nose. Scarlet fever-like rash with desquamation. Pastia lines present.

Generalized. Most marked on trunk

Fever, pharyngitis, and cervical lymphadenitis. Rash onset within 2 days of first symptoms. Incubation period 3-4 days

Diffuse erythematous and fine maculopapular (looks and feels like red sandpaper). Rash darker in skin folds (Pastia lines). Desquamation occurs. Rose spots. 2-4 mm macular lesions

Circumoral pallor. Generalized rash, with trunk and proximal end ofextremities being most involved. Discrete lesion on abdomen

Fever and pharyngitis, Sudden onset of rash

Characteristic rash is petechial or purpuric. Early lesions may be erythematous, maculopapular, or urticarial

Generalized

Fever, conjunctivitis, and anorexia. Rash rarely noted 5 days or more of high fever, sore throat, cervical lymphadenopathy, mucosal erythema, conjunctivitis without exudates, skin rash, and desquamation of the skin on the fingers and toes

Erythematous maculopapular rash Maculopapular rash

Mainly on trunk Generalized

4–7

Prepubertal children & Adults

7-17

Parvovirus B19

INFECTIOUS MONONUCLEOSIS

Epstein-Barr Virus

Children & Adolescents

28-49

Hepatitis B

1 – 6 yr

50 – 180 14 - 60

Neonates & infants

SCARLET FEVER

Streptococcus pyogenes

School age

TYPHOID FEVER

Salmonella typhi

Older children & adults

MENINGOCOCCEMIA

N. Meningitidis

Any (<5 yr)

LEPTOSPIROSIS

Leptospira spp.

KAWASAKI DISEASE

Unknown

Starts behind ears and on forehead. Spreads downward over body. Confluence most prominent on face, trunk, and proximal end of extremities Starts on face and spreads downward to trunk and extremities

Erythematous, maculopapular, and confluent. Develop a brownish appearance, and fine desquamation occurs

Infants & young children

Staphylococcus aureus (exfoliative toxinproducing)

DISTRIBUTION

Onset with fever, cough, coryza, and conjunctivitis. About 2 days after onset, appearance of enanthem (Koplik spots), and 2 days later, onset of exanthem

6 mos – 2 yrs

STAPHYLOCOCCAL SCARLATINIFORM ERUPTION SSSS, TEN (Ritter or Lyell’s Syndrome)

LESIONS

8 – 12

ERYTHEMA INFECTIOSUM

PAPULAR ACRODERMATITIS OF CHILDHOOD HUMAN IMMUNEDEFICIENY VIRUS

CLINICAL CHARACTERISTICS

Fever, pharyngitis, and lymphadenopathy. Exanthem occurs in 3-13% of cases. If ampicillin administered, then exanthem in 50% of cases Insidious onset with arthralgia, arthritis, and rash occurring before jaundice

Malaise, headache, and marked fever. Rash onset 10 days after onset of fever

Most commonly erythematous, maculopapular, and discrete. May have macular, petechial, vesicular, and urticarial components. Rarely erythema multiforme

Most prominent of neck and trunk. Face and extremities may be affected Usually starts on face and spreads downward to trunk and extremitities. May have peripheral distribution (hand, foot, and mouth syndrome)

Mainly on trunk and proximal end of extremities

Generalized Mainly chest and abdomen

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3 CLINICAL COURSE

RASHES

OTHER SYMPTOMS

Measles

Conjuctivitis with photophobia Koplik’s spots

Rubella

“Blueberry Muffin” Lesion

Roseola infantum

Scarlet fever

Typhoid

Rose Spots

Stepladder Appearance

Kawasaki Disease