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Do we need to distinguish kung EM Minor or Major ung patient?

ERYTHEMA MULTIFORME

Erythema MultiformeEM minor & EM with mucosal involvement• Self-limited, recurrent disease• No or only a mild prodrome (1 to 4 weeks)• Sharply marginated erythematous macules

become raised, edematous papules (24 to 48 hours)

• Koebner’s phenomenon or photoaccentuation• Mucosal involvement in 25%

-- usually limited to the oral mucosa• More severe classic case? Two or more

mucous membranes involved in 45%

EM Minor

• Periphery: ring of erythema

• Central: flatters, more pruritic and dusky

• “target” or “iris” lesion with three zones

Characteristic & Evolution of the Lesion

1. Central dusky purpura2. Elevated, edematous, pale ring3. Surrounding macular erythema

EM Minor

Sites of Predilection(Symmetrical and acral)• (Best observed on)

Palms and soles• Dorsal feet• Extensor limbs• Elbows• Knees

Age of Predilection• young adults

Erythema Multiforme

Steven-Johnson syndrome / EM major• Clinically different from minor• Frequently, febrile prodrome

EM Major

• Flat, erythematous or purpuric macules

incomplete “atypical targets” (may blister centrally

• Larger and more commonly confluent lesions compared to EM minor

Characteristic & Evolution of the Lesion

EM MajorSites of Predilection• Begins diffusely on the

trunk and mucous membranes

• Spreads centripetally

Age of Predilection• Eruption occurs at all ages

Etiologic Factors

• EM minor = herpes simplex infection– Typically orolabial– 1 to 3 weeks (10 day average) after herpes lesion– May or not follow herpes outbreaks

• EM major (SJS) = medications– Most centrally accentuated eruptions with atypical targets – Sulfonamides, antibiotics, NSAIDs, allopurinol,

anticonvulsants– Due to abnormal metabolism of medications

Etiologic Factors

• Also, EM major = Mycoplasma pneumoniae– Prominent mucosal involvement and bullous skin

lesions – NOT classic iris lesions– Resemble SJS cases

• And, EM major = radiation therapy– With phenytoin and tapering corticosteroids –

induces EM starting at radiation port

Pathogenesis

• Activated T lymphocytes – Epidermis: cytotoxic or suppressor cells – Dermis: helper T cells

• EM minor – specific HLA types (HLA-DQ3)• SJS – abnormalities in drug metabolism

Hence, there is a genetic component for both diseases

Disease

• Physical examination– Characteristic Target Lesions– Distribution- symmetrical and acral– Evolution:

Diagnosis

Ring of Erythema

Lesions flatten at the center

Center becomes darker and purpuric

Erythema Multiforme Salient FeaturesEM Minor: Young adultsEM Major: Eruption occurs at all ages

25 year old female

•Sharply marginated erythematous macules•Become raised edematous papules in 24 to 48 hours•Central area may darken and form blisters

Multiple erythematous papules, macules and patches with dark centers5 days duration Appearance of multiple pruritic macules and papules after 2 days

sites of predilection•Dorsum of hands•Dorsum of feet•Extensor limbs•Elbows•Knees•Palms•Soles •Trunk

Flexor surface of both forearms which gradually spread to the face, trunk and thighs, palms and soles