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February 1973

Progressive, Atrophying, Chronic Granulomatous DermohypodermitisAutoimmune Disease?

Author Affiliations

Caracas, Venezuela

From the Instituto Nacional de Dermatologia, Caracas, Venezuela.

Arch Dermatol. 1973;107(2):271-274. doi:10.1001/archderm.1973.01620170079022

A chronic, progressive dermatosis is described, characterized by papular lesions forming plaques in various parts of the body, with secondary phenomena of acquired cutis laxa. Histologically, the lesions were typified by a granuloma formed by histiocytes, epitheliod cells and giant cells, lymphocytes, plasmocytes, and lipophagia phenomena.

The disease started after the successive injection of Mitsuda antigen and of BCG vaccine. One of the initial lesions appeared at the site where the Mitsuda antigen had been injected. The disease evolved during a period of more than 20 years.

Oral treatment with corticosteroid produced partial regression. When treatment with this drug was discontinued the clinical manifestations became worse, with fever and arthralgia.

In face of such a special clinical picture we thought of the possibility that it might be an autoimmune disease. When azathioprine was administered, there was regression of the lesions.

The patient died after a period of intense diahrrea. He also had a generalized inflammation of lymph nodes. The postmortem study of one of his lymph nodes rendered a diagnosis of Hogdkin's disease.