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).
