Blistering/Papular diseases Flashcards
PEMPHIGUS VULGARIS
PEMPHIGUS VULGARIS
PEMPHIGUS VULGARIS
PEMPHIGUS VULGARIS
general
▸ Life-threatening, chronic autoimmune blistering disorder of the mucus membranes and skin
▸ Most common in patients in their 30s and 40s
▸ Associated with several medications – penicillamine, captopril, cephalosporins, phenobarbital
(PPCC)
PEMPHIGUS VULGARIS
pathophys
Type of sensitivity
▸ Type II hypersensitivity reaction where autoantibodies against desmoglein lead to
acantholysis
▸ Desmoglein connects keratinocytes in the skin
PEMPHIGUS VULGARIS
Clin man
▸ Painful erosion or ulceration initially, followed by painful, flaccid skin bola that rupture easily, leaving painful
denuded skin erosions that bleed easily.
▸ Initial lesion is most commonly intraoral
▸ Positive Nikolsky sign – detachment of skin under pressure/trauma
PEMPHIGUS VULGARIS
Dx and Tx
Diagnosis: punch biopsy – IgG throughout the epidermis, basal keratinocytes in a pattern that resembles “a row of
tombstones”
▸ ELISA - anti-desmoglein or anti-epithelial autoantibodies
▸ Treatment: refer to dermatology
▸ Mainstay of treatment: Systemic glucocorticoids + wound care +/-rituximab, mycophenolate or azathioprine
BULLOUS PEMPHIGOID
BULLOUS PEMPHIGOID
BULLOUS PEMPHIGOID
general
Induced by
▸ Autoimmune disorder leading to blister formation
and severe itching
▸ Primarily seen in the elderly
▸ Can be drug induced – loop diuretics, metformin
bullous pemphigoid
pathophys and type of sensitivity
Type II hypersensitivity reaction – IgG
autoantibodies against hemidesmosomes and
basement membrane zone causing subepidermal
blistering
BULLOUS PEMPHIGOID
Clin man
▸ Prodrome of pruritus with eczematous or urticarial erythematous plaques followed by multiple tense large bullae that easily rupture
▸ Most commonly found in the groin, axilla, trunk, flexural areas of the extremities
▸ Mucosal disease in 10-30%
▸ Negative Nikolsky sign
bullous pemphigoid
Dx and Tx
Mild/localized and severe
Diagnosis: punch biopsy with direct immunofluorescence - linear C3, IgG along the dermal-epidermal junction, subepidermal blistering,
eosinophilia
▸ ELISA: autoantibodies against BP antigen 230 & 180
Treatment: refer to dermatology
▸ Localized/Mild - High potency topical corticosteroids + tetracyclines (doxycycline)
▸ Extensive/Severe - systemic corticosteroid
PORPHYRIA CUTANEA TARDA
PORPHYRIA CUTANEA TARDA
general
Hypersensitivity of the skin to abnormal porphyrins when exposed to light, leading to a blistering disease of sun exposed areas
▸ Enzyme: uroporphyrinogen decarboxylase