Table 1.

Clinical diagnosis and laboratory work-up of pemphigus diseases; hematoxylin and eosin (H&E), direct immunofluorescence (DIF), indirect immunofluorescence (IIF).

	Clinical Presentation	H&E	DIF	IIF	Serology
Pemphigus vulgaris	Painful blisters and erosions predominating in oropharyngeal mucous membranes; symptoms can include dysphagia, vocal hoarseness, vaginal irritation, painful sexual intercourse; palms and soles are spared	Suprabasilar acantholysis with retention of basal keratinocytes along the basement membrane (“tombstoning”)	Intercellular deposition of immunoglobulin G (IgG)	Intercellular deposition of immunoglobulin G (IgG) circulating antibodies Utilize monkey esophagus substrate	Presence of antibodies against both desmoglein 1 and desmoglein 3 or antibodies against desmoglein 3 only
Pemphigus foliaceus	Painful blisters and erosions without mucosal membrane involvement; cutaneous involvement primarily concentrated in seborrheic areas (scalp, face, upper trunk)	Acantholysis within upper epidermis, adjacent or within the granular layer, leading to a subcorneal cleft If significant eosinophils are present, consider drug-induced pemphigus	Intercellular deposition of immunoglobulin G (IgG) Negative DIF studies are not uncommon in drug-induced pemphigus	Intercellular deposition of immunoglobulin G (IgG) circulating antibodies Utilize guinea pig esophagus substrate	Presence of antibodies against desmoglein 1 only
Paraneoplastic pemphigus	Severe mucosal involvement and polymorphous skin lesions with associated underlying malignancy or neoplasm (e.g. Non-Hodgkin’s lymphoma, chronic lymphocytic leukemia, Castleman’s disease)	Suprabasilar acantholysis, keratinocyte necrosis, and interface change	Intercellular deposition of immunoglobulin G (IgG) Negative DIF studies are not uncommon in paraneoplastic pemphigus	Intercellular deposition of immunoglobulin G (IgG) circulating antibodies Utilize rat bladder substrate	Presence of antibodies against plakin proteins
IgA pemphigus	Tense bullae that transition into clear fluid-filled blisters; cutaneous involvement of vesicles (81%), pustules (75%), and erythematous annular plaques (64%) primarily seen in flexural areas of proximal extremities and trunk; mucous membranes usually spared	Subcorneal pustular dermatosis type: subcorneal vesiculopustules with minimal acantholysis Intraepidermal neutrophilic dermatosis type: intraepidermal vesiculopustules with variable acantholysis	Intercellular deposition of immunoglobulin A (IgA)	Intercellular deposition of immunoglobulin A (IgA) circulating antibodies	Subcorneal pustular dermatosis type: presence of antibodies against desmocollin 1 Intraepidermal neutrophilic dermatosis type: presence of IgA antibodies against desmoglein 1 and 3