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

Skin lesions at various stages of evolution

VARICELLA

“dew drop-like lesions”

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 Dengue transmitted by

infected female mosquitoinfected female mosquito

Primarily a daytime feederPrimarily a daytime feeder

Lives around human Lives around human

habitationhabitation

Lays eggs and produces Lays eggs and produces

larvae preferentially in larvae preferentially in

artificial containers with clean artificial containers with clean

stagnant waterstagnant water

Undifferentiated fever – may be the Undifferentiated fever – may be the

most common presentation*most common presentation*

Classic dengue feverClassic dengue fever

Dengue hemorrhagic fever (DHF)Dengue hemorrhagic fever (DHF)

Dengue shock syndrome (DSS)Dengue shock syndrome (DSS)

Dengue Clinical PresentationsDengue Clinical Presentations

In infants and young childrenIn infants and young children

– Undifferentiated febrile illnessUndifferentiated febrile illness

– Maculopapular rashMaculopapular rash

– Petechiae and a positive tourniquet test, Petechiae and a positive tourniquet test,

not uncommonnot uncommon

Clinical Characteristics of Dengue Fever Clinical Characteristics of Dengue Fever

Clinical Characteristics of Dengue Fever Clinical Characteristics of Dengue Fever

Older childrenOlder children

– Mild febrile illness, orMild febrile illness, or

– High fever withHigh fever with

Severe headacheSevere headache

Pain behind the eyesPain behind the eyes

Muscle and joint painsMuscle and joint pains

RashRash

Petechiae and a positive tourniquetPetechiae and a positive tourniquet test test

Fever, or recent history of acute feverFever, or recent history of acute fever

Hemorrhagic manifestationsHemorrhagic manifestations

Low platelet count (100,000/mmLow platelet count (100,000/mm33 or less) or less)

Objective evidence of “leaky capillaries:”Objective evidence of “leaky capillaries:”

elevated hematocrit (20% or more over baseline)elevated hematocrit (20% or more over baseline)

low albuminlow albumin

pleural or other effusionspleural or other effusions

Clinical Case Definition forClinical Case Definition forDengue Hemorrhagic FeverDengue Hemorrhagic Fever

4 Necessary Criteria4 Necessary Criteria

4 criteria for DHF4 criteria for DHF

Evidence of circulatory failure manifested Evidence of circulatory failure manifested

indirectly by all of the following:indirectly by all of the following:

Rapid and weak pulseRapid and weak pulse

Narrow pulse pressure (Narrow pulse pressure (20 mm Hg) 20 mm Hg) OR OR

hypotension for agehypotension for age

Cold, clammy skin and altered mental statusCold, clammy skin and altered mental status

Frank shock is direct evidence of circulatory Frank shock is direct evidence of circulatory

failurefailure

Clinical Case Definition for Clinical Case Definition for Dengue Shock SyndromeDengue Shock Syndrome

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)

Grade Manifestations

I FeverNon-specific constitutional symptomssuch 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

Dengue Hemorrhagic FeverDengue Hemorrhagic FeverGrading the SeverityGrading the Severity

Skin hemorrhages: Skin hemorrhages:

petechiae, purpura, ecchymosespetechiae, purpura, ecchymoses

Gum bleedingGum bleeding

Nose bleedingNose bleeding

Gastro-intestinal bleeding: Gastro-intestinal bleeding:

hematemesis, melena, hematocheziahematemesis, melena, hematochezia

HematuriaHematuria

Increased menstrual flowIncreased menstrual flow

Hemorrhagic Manifestations of DengueHemorrhagic Manifestations of Dengue

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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