(Figure 3.25C) - Associated with underlying benign or malignant neoplasm: non-Hodgkin’s lymphoma (most common), chronic lymphocytic leukemia, Castleman’s disease, thymoma, Waldenström macroglobulinemia, sarcoma
| | | | | | Two benign diseases: Castleman’s disease and benign thymoma | | | | | |
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- Autoantigen: various desmosomal proteins including desmoglein 3, periplakin, envoplakin, desmoplakin 1/2, BPAG1, plectin, 170 kDa Ag, rarely desmoglein 1
- Clinical: severe stomatitis that extends onto vermilion lip; intraorally can involve entire oropharynx; cutaneous presentation varies considerably from lichenoid papules, flaccid or tense bullae, erythematous macules to erythema-multiforme-like lesions on hands/ft; conjunctivae, perianal and genital region may develop painful erosions
- Histology: suprabasilar acantholysis, ± vacuolar basal layer damage associated with lichenoid dermal lymphocytic infiltrate, and necrotic keratinocytes
- DIF: intercellular + linear IgG/C3 along BMZ
- IIF: intercellular IgG, best substrate is rat bladder epithelium (most sensitive)
- Treatment: stomatitis refractory to treatment and immunosuppressive agents less effective than for PV; prognosis associated with underlying neoplasm
| | | | | | Binds rat bladder transitional epithelium unlike PV | | | | | |
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| | Figure 3.25
A: DIF, pemphigus vulgaris
(Courtesy of Dr. Paul Getz)
B: Pemphigus foliaceous
(Courtesy of Dr. Paul Getz)
C: Paraneoplastic pemphigus |
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