Disorder | Epidemiology | Clinical features | Diagnosis | Treatment | Comments |
---|---|---|---|---|---|
Pemphigus vulgaris | Rare, equal in men and women, onset at 40 to 60 years of age, more common in Jewish or Mediterranean descendants | Flaccid blisters or crushed erosions, located on head, upper trunk, intertriginous areas, and mucosa; often begins on oral mucosa, may lead to hoarseness; skin is usually painful and Nikolsky's sign positive | Light: suprabasilar blister with acantholysis DIF: intercellular IgG and C3 | Corticosteroids (prednisone, 1 mg per kg per day) taper to maintenance; may add azathioprine (Imuran), methotrexate, cyclophosphamide (Cytoxan), mycophenolate mofetil (CellCept); plasmapheresis | May be fatal if untreated, risk of infection in treated and untreated cases |
Paraneoplastic pemphigus | Very rare, female to male ratio is 2:1, onset at 60 years and older, associated with malignancy, most often lymphoid | Extensive painful mucocutaneous erosions, resembling pemphigus vulgaris, in the presence of neoplasm; targetoid erythematous papules with dusky centers resembling erythema multiforme may also be present | Light: suprabasilar blister with acantholysis (PV-like), with basal cell vacuolation, exocytosis, and dyskeratotic keratinocytes (erythema multiforme-like) | Detection and treatment of the neoplasm | Usually rapidly fatal if associated with a malignant neoplasm |
DIF: intercellular and basement membrane IgG and C3 | |||||
IIF: positive for rat bladder (cuboidal epithelium) and stratified squamous epithelium | |||||
Bullous pemphigoid | Equal in men and women, onset at 60 to 80 years of age, rarely in children | Tense bullae with clear fluid or erosions, may begin as erythematous urticarial, pruritic plaques, localized or generalized on the lower legs, forearms, thighs, groin, abdomen, but rarely on mucosa | Light: subepidermal blister with mixed superficial inflammation DIF: IgG and/or C3 at the basement membrane | Corticosteroids (prednisone) alone or with azathioprine, mycophenolate mofetil or a tetracycline | Self-limited, rarely fatal even if untreated |
Cicatricial pemphigoid | Rare, female to male ratio is 2:1, onset at 40 to 60 years of age | Bullae rupture within hours leaving painful erosions that heal with scarring; most common sites are oral and conjunctival mucosa; skin involved in one third of cases, head and upper chest | Light: subepidermal blister with mixed infiltrate in fresh lesions and predominant fibroblasts in older, scarring lesions | Corticosteroids; may add dapsone for oral involvement or cyclophosphamide or azathioprine for ocular involvement; topical treatment of oral lesions with steroid gel or dexamethasone mouthwash (Roxane) | Ocular lesions may lead to blindness; oral complications include laryngeal or esophageal strictures with dysphagia, hoarseness, loss of voice, or need for tracheostomy |
DIF: linear IgG and C3 on the basement membrane, +/− IgA or IgM treatment of oral lesions | |||||
Dermatitis herpetiformis (dapsone) | Rare, male to female ratio is 2:1, onset at 20 to 40 years of age, but also in children, whites, rare in blacks or Asians | Grouped (herpetiform) excoriations or vesicles symmetrically located on extensor remission; watch for signs surfaces of elbows, knees, sacrum, buttocks, and shoulders with intense pruritus and burning sensation | Light: neutrophilic abscesses in dermal papillae, dermal infiltrates of neutrophils and eosinophils with subepidermal vesicles | Gluten-free diet; sulfones dermal infiltrates of neutrophils and eosinophils | Chronic with occasional remission; watch for signs of other autoimmune disorders; may coexist with gluten-sensitive enteropathy |
DIF: granular IgA deposits in the tips of the dermal papillae | |||||
Linear IgA dermatosis (LAD) or chronic bullous disease of childhood (CBDC) | LAD: equal in men and women, onset after puberty, usually in 30s | LAD: pruritic symmetric grouped annular crusted papules, vesicles, or bullae on extensor surfaces, elbows, knees, or buttocks | Light : subepidermal bullae with infiltrate of neutrophils at basement membrane and dermal papillae tips | Sulfones (dapsone) +/− low-dose prednisone | LAD: variable course |
CBDC: onset before age five | CBDC: rings of grouped bullae around old lesions on genitalia, face, or perioral area | DIF: linear deposits of IgA along the basement membrane, +/− IgG and C3 | CBDC: usually clears in two years or less |