Anti-epiligrin cicatricial pemphigoid (AECP) is a chronic, mucous membrane-dominated, subepithelial blistering disease characterized by circulating anti-basement membrane zone auto-antibodies to laminin 5. Recent studies have shown that people with AECP have an increased relative risk for malignant tumours. In this report we describe two patients with AECP. In both cases, indirect immunofluorescence demonstrated circulating IgG anti-basement membrane auto-antibodies that bound to the dermal side of 1M NaCl split normal skin. Immunoblotting using laminin 5 purified from keratinocyte extract as a substrate showed that the IgG antibodies of patient 1 reacted with the 140-kDa beta3 subunit of laminin 5 and IgG antibodies of patient 2 reacted with the 165-kDa and 145-kDa alpha3 subunits. Patient 1 had prostate carcinoma and his blistering was resistant to therapy. Patient 2 had no detectable malignancy and treatment with doxycycline was successful.
Muir-Torre syndrome (MTS) is an autosomaldominant skin condition of genetic origin, characterized by tumors of the sebaceous glands or keratoacanthomas that are associated with malignant visceral diseases. MTS associated with gynecologic malignancy has rarely been reported. Here we report a woman with no family history of colorectal cancer who developed endometrial carcinoma, stage 3a, at 49 years of age and at age 51 years, developed two skin tumors, a nasal squamous cell carcinoma and a sebaceous carcinoma of the right eyelid. The appearance pattern of these skin tumors suggested MTS. Although MTS associated with endometrial carcinoma is rare, patients with endometrial carcinoma should undergo evaluation for visceral malignancies (mainly colon cancer) and sebaceous skin lesions, regardless of whether or not there is a family history of colorectal cancer.
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