Version October 2024
| Disorder | Clinical features | Pathology | Laboratory findings |
| Bullous pemphigoid | Tense bullae, urticarial papules and plaques; mucous membrane involvement in up to 30% of cases | H&E: subepidermal blister with eosinophils; DIF: linear basement membrane zone deposition of IgG and C3 | IIF: IgG anti-BP antigen 180 and 230 |
| Pemphigus vulgaris | Flaccid vesicles and erosions on skin and mucous membranes, desquamative gingivitis; scalp involvement common | H&E: intraepidermal blister formation, acantholysis; DIF: intercellular epidermal IgG deposition involving lower epidermis | IIF: intercellular IgG staining of skin substrate, anti-desmoglein 3 IgG |
| Pemphigus foliaceus | Flaccid vesicles and erosions; no mucous membrane involvement | H&E: intraepidermal vesicle and acantholysis (blister is subcorneal); DIF: intercellular IgG deposition in upper epidermis | IIF: intercellular IgG staining of skin substrate; anti-desmoglein 1 IgG |
| Stevens-Johnson syndrome and toxic epidermal necrolysis | Exudative erosions of lips, oral mucosa, eyes, genital mucosa; targetoid papulovesicles of skin, skin sloughing, and skin pain | H&E: vacuolar interface dermatitis or epidermal necrosis | None |
| Paraneoplastic pemphigus | Targetoid papulovesicles, erythema multiforme-like cutaneous lesions; exudative erosions of lips, oral mucosal, eyes, genital mucosa | H&E: mixed histology with overlapping features of pemphigus vulgaris, erythema multiforme, and lichen planus; DIF: staining of transitional epithelium (rodent bladder) | IIF: antibodies to multiple antigens (desmoplakins, desmogleins, bullous pemphigoid antigen 1, etc) |
| Dermatitis herpetiformis | Grouped papulovesicles on elbows, knees, buttocks, scalp; intense pruritus | H&E: subepidermal blister with neutrophils in dermal papillae; DIF: IgA in dermal papillae | Elevated serum IgA antiepidermal transglutaminase and tissue transglutaminase antibodies |
| Disseminated HSV/VZV | Fever, hepatitis, CNS involvement; grouped and scattered monomorphic vesicles | Intraepidermal blister; ballooning of keratinocyte cytoplasm and margination of chromatin to form intranuclear inclusion bodies; mixed inflammatory infiltrate | Viral culture, Tzanck smear, DFA, PCR |
| Linear IgA bullous dermatosis | Annular or arcuate vesicles and bullae; can have mucous membrane involvement | H&E: subepidermal blister with predominance of neutrophils; DIF: linear IgA along basement membrane zone | IIF: circulating IgA antibasement membrane antibodies |
| Epidermolysis bullosa acquisita | Tense blisters and erosions with scarring and milia | H&E: subepidermal blisters with mixed inflammatory infiltrate of neutrophils, eosinophils, lymphocytes; DIF: broad, linear IgG along the basement membrane zone; in salt-split skin, IgG stains dermal side | |
| Staphylococcal scalded skin syndrome | Widespread erythema, flaccid bullae, erosions, desquamation of skin | H&E: subcorneal blister (split within the granular cell layer) | Toxin-mediated condition; culture often negative at sites of blistering |
| Bullous systemic lupus erythematosus | Other skin findings of SLE: nail fold telangiectasias, malar erythema, discoid or subacute cutaneous lupus | H&E: similar to dermatitis herpetiformis; DIF: mixed features similar to bullous pemphigoid and lupus | Antinuclear antibody test |
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