PARANEOPLASTIC PEMPHIGUS
PARANEOPLASTIC PEMPHIGUS (See also Section 19) ICD-10: L10.81
• Painful mucosal erosions, stomatitis; cutaneous blisters, lichenoid papules and erosions; conjunctival erythema can be prominent (see Fig. 33-25). Cutaneous manifestations are variable: Bulla, lichenoid, erythema multiforme-like.
• Confirmed or occult malignancy (though this may precede or lag presentations by 6 months to a year). Can be associated with bronchiolitis obliterans-like pulmonary defects.
• Acantholysis, keratinocyte necrosis, and interface dermatitis. IgG and complement (C3) within the epidermal intercellular spaces and basement membrane seen on immunofluorescence. Circulating antibodies specific for stratified or transitional epithelium can be drawn from blood (ELISA, immunoprecipitation).
• Treatment is generally systemic; topical treatments are symptomatic.
FIGURE 33-25 • Paraneoplastic pemphigus Note beefy-red erosive mucositis in this patient with advanced CLL. (Used with permission from Dr. Mark Lerman.)