Paraneoplastic pemphigus may affect the skin and mucous membranes in patients with a variety of benign and malignant neoplasms.


Clinical features:

(1) polymorphous skin lesions, with erythema, blisters, vesicles, macules, papules and erosions

(2) lesions of the oral mucosa and lips, especially erosions and ulcerations

(3) variable conjunctivitis with scarring (cicatrization)

(4) variable involvement of other mucosal surfaces (gastrointestinal tract, respiratory tract, etc)


Associated tumors:

(1) CLL, non-Hodgkin's lymphoma, Castleman's disease or other hematologic malignancy

(2) squamous cell carcinoma, especially of the lung

(3) adenocarcinoma

(4) malignant melanoma

(5) sarcoma

(6) benign soft tissue tumors

(7) thymoma


The onset of lesions may precede the diagnosis of the underlying tumor.


Autoantibodies that may be present in the serum:

(1) anti-desmoplakin I

(2) anti-desmoplakin II

(3) anti-periplakin

(4) anti-envoplakin


Immunofluorescence: linear deposition of IgG and C3 along the basement membrane (subepithelial) and between epithelial cells (intercellular).


To read more or access our algorithms and calculators, please log in or register.