Vesiculobullous, Numerous Lesions

Fig. 13.1 Bullous pemphigoid versus pemphigus vulgaris. A,B Tense blisters typical of bullous pemphigoid. The blisters are located within pink, urticarial plaques. C,D Flaccid blisters typical of pemphigus vulgaris. The base of the blister is non-erythematous. A, Courtesy, Yale Dermatology Residents’ Slide Collection.

Fig. 13.2 Clustered vesicles/bullae. A Dermatitis herpetiformis. Clustered small vesicles (“herpetiform”) and erosions. B Linear IgA disease. Clusters of larger vesicles/bullae in annular arrangements. Courtesy, Yale Dermatology Residents’ Slide Collection.

Table 13.2

Blistering disorders – level of the split

Level of split	Blistering disorder
Subepidermal	Bullous pemphigoid Pemphigoid gestationis Cicatricial pemphigoid Epidermolysis bullosa acquisita Porphyria cutanea tarda Linear IgA disease Dermatitis herpetiformis Dystrophic epidermolysis bullosa Bullous lupus erythematosus Bullous lichen planus
Intraepidermal	Pemphigus foliaceus Pemphigus vulgaris Herpes virus infections (eczema herpeticum, disseminated zoster, chickenpox) Hand-foot-and-mouth disease

Table 13.3

Blistering disorders – usual direct immunofluorescence patterns

Direct immunofluorescence pattern	Blistering disorder
Linear C3 and IgG at the dermal–epidermal junction	Bullous pemphigoid Pemphigoid gestationis Cicatricial pemphigoid Epidermolysis bullosa acquisita Bullous lupus erythematosus*
Intercellular IgG and C3	Pemphigus vulgaris Pemphigus foliaceus
Linear IgA at the dermal–epidermal junction	Linear IgA disease Cicatricial pemphigoid
Granular IgA in the papillary dermis	Dermatitis herpetiformis

Table 13.4

Blistering disorders – antibody profiles

Blistering disorder	Antibodies directed against
Bullous pemphigoid	BPAg1, BPAg2
Pemphigus vulgaris	Desmoglein 3
Pemphigus foliaceus	Desmoglein 1
Dermatitis herpetiformis	Epidermal transglutaminase
Linear IgA	BPAg2
Cicatricial pemphigoid	BPAg2
Pemphigoid gestationis	BPAg2
Epidermolysis bullosa acquisita	Type VII collagen
Bullous lupus erythematosus	Type VII collagen

This chapter focuses on bullous pemphigoid, pemphigoid gestationis, cicatricial pemphigoid, epidermolysis bullosa acquisita, porphyria cutanea tarda, linear IgA disease, dermatitis herpetiformis, bullous lupus erythematosus, bullous lichen planus, pemphigus, herpes, and coxsackie virus infection.