Chie Nakatani

A Case of Nonscarring Inflammatory Epidermolysis Bullosa Acquisita: Characterization of IgG Autoantibodies by Immunofluorescence, Immunoblotting and Immunogold Electron Microscopy

We report a case of nonscarring inflammatory epidermolysis bullosa acquisita in a 59‐year‐old Japanese woman. She developed blisters and erosions on her lip, trunk and extremities. Sodium aurothiomalate was effective for the skin lesions. The patient had been free from bullous skin lesions for the last 13 years and had shown no scarring. Indirect immunofluorescence (IF) study on 1 M NaCl‐split skin revealed IgG autoantibodies against the dermal side of the split skin. Immunoblotting using normal human dermal extracts disclosed IgG autoantibodies reactive with the 290 and 145 kD antigens. Circulating IgG autoantibodies were deposited on the lamina densa by immunoelectron microscopy. IF mapping using several antibodies for the components of the basement membrane zone revealed blister formation at the lamina densa. These results suggest that the cleavage at the lamina lucida does not necessarily exclude the diagnosis of EBA and that the definite diagnosis of EBA should be confirmed by immunoblotting or immunoelectron microscopic study.

LOCALIZED PRETIBIAL PEMPHIGOID ASSOCIATED WITH TRICHILEMMAL CARCINOMA

We report a case of localized pretibial pemphigoid with concurrent trichilemmal carcinoma of the face in a 93‐year‐old woman. Immunoblot analysis showed circulating autoantibodies against the 230 kDa antigen. After removal of the skin tumor, the bullous skin lesions were reduced and healed with only the topical application of corticosteroid.

Association of IgA immune complex vasculitis and rheumatoid arthritis

had a single 3 X 4 mm lesion on the upper part ofthe right helix. Our patient was much older and had multiple small lesions on both helixes. The differential diagnosis includes elastotic nodules of the ears, chondrodermatitis nodularis helices, polychondritis, keloids, auricular tophi, granuloma annulare, sarcoidosis, and pinnal calcifications. These were easily excluded clinically and histopathologically. Hamartomas may occur in any organ but are most common in the viscera. The origin of hamartomas of the skin is not well understood. According to Willis,3 the term hamartoma should be applied only to lesions for which there is definite evidence of a developmental anomalyeither actual malformation, with tissue excess already present at birth, or an inborn tissue anomaly that is manifested by excessive growth until the patient reaches puberty. These hamartomas may arise from cell nests that formed during embryonal life and may possess the potential to develop into tumorlike malformations. Several factors may be important in the clinical manifestation of harmatomas of the skin. These may include repeated friction and long-term sun exposure. Delayed appearance ofharmatomas, particularly in our patient, is unusual. Continuous auricular growth after physical maturity, with subsequent tightening of the skin caused by the prominence of underlying cartilage as human beings age,4 may also act as a precipitating factor. The treatment of skin hamartomas is usually surgical, but if the tumor is not excised with clear margins, it may recur. In our patient, one lesion had been excised 2 years previously and had not recurred. The patient of Kacker and Dasgupta2 showed no reappearance 7 years after surgical removal.