Paraneoplastic Pemphigus (PNP)


(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
     
       

  • 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
     
       

Figure 3.25 A: DIF, pemphigus vulgaris (Courtesy of Dr. Paul Getz) B: Pemphigus foliaceous (Courtesy of Dr. Paul Getz) C: Paraneoplastic pemphigus
Figure 3.25
A: DIF, pemphigus vulgaris
(Courtesy of Dr. Paul Getz)
B: Pemphigus foliaceous
(Courtesy of Dr. Paul Getz)
C: Paraneoplastic pemphigus