[Cutaneo-systemic papulosclerotic mucinosis (scleromyxedema): remission after extracorporeal photochemotherapy and corticoid bolus]. / Mucinose papuloscléreuse (scléromyxoedème) cutanéo-systémique: rémission après photochimiothérapie extracorporelle et bolus de corticoïdes.

INTRODUCTION: Scleromyxedema is the consequence of a dermal infiltration by mucine, commonly associated with a monoclonal gammapathy of unknown significance. CASE REPORT: A 46 year-old woman was hospitalized for a scleromyxedema with a bilateral macular edema and a restricted pulmonary syndrome. A quite complete cutaneous response and a complete ocular and pulmonary response were obtained after 12 extracorporeal photopheresis courses and 4 flashes of prednisolone (17 months follow-up). COMMENTS: There is no consensus on guidelines for the treatment of scleromyxedema. Steroids and melphalan are usually indicated. However these drugs induce severe side-effects. In the absence of controlled studies concerning the efficiency of the different drugs used in scleromyxedema, the main advantage of photopheresis lies in the safety of this procedure.

[The Waardenburg-Klein syndrome]. / Le syndrome de waardenburg-klein

A case of Waardenburg-Klein syndrome associated with a Hodgkin's disease is reported in a 29 year old female. Such an association seems to be fortuitous. The authors discuss the dermatological aspects of this rare disease, particularly the disturbances of cutaneous pigmentation. Ultrastructural study of depigmented skin indicates the melanocytes are absent and precise the place of this depigmentation in the group of genetic abnormalities of skin pigmentation.