A CASE OF GRANULOMATOUS SLACK SKIN IN A C HILD - LITERATURE-REVIEW AND UPDATE ON CUTANEOUS LYMPHOMAS IN CHILDREN

This report describes a pediatric case of granulomatous slack skin, wh ich is a slowly-evolving granulomatous primary lymphoma of the skin. D iscrete skin lesions resembling pityriasis lichenoides first appeared at three years of age and were followed by multiple, soft, infiltrated tumors that progressed to scarred, atrophic, pendulous lesions. Splen omegaly, lymphadenopathy, and ophthalmic and ENT involvement were addi tional features. Skin biopsies showed infiltrates composed of small ro und cells and multinucleate giant cells, and biopsies of other organs demonstrated tuberculoid granulomas. The disease ran a slowly progress ive course, and the patient died at 21 years of age with severe sepsis complicating disseminated large cell T-cell lymphoma. Primary cutaneo us lymphomas are exceedingly rare in children, and this is the first r eported case occurring at such a young age. This case illustrates the diagnostic problems raised by lymphomas in children and the diagnostic usefulness of immunohistochemistry and gene rearrangement studies, It also provides an opportunity for discussing potential links between l ymphoma and sarcoidosis.