[Cutaneo-systemic papulosclerotic mucinosis (scleromyxedema): remission after extracorporeal photochemotherapy and corticoid bolus].

Abstract

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.

• Authors: M D'Incan, F Franck, J Kanold, F Bacin, R Achin, A J Beyvin, F Demeocq, P Souteyrand
• Journal: Annales de dermatologie et de venereologie (Ann Dermatol Venereol) Vol. 128 Issue 1 Pg. 38-41 (Jan 2001) ISSN: 0151-9638 [Print] France
• Vernacular Title: Mucinose papuloscléreuse (scléromyxoedème) cutanéo-systémique: rémission après photochimiothérapie extracorporelle et bolus de corticoïdes.
• PMID: 11226899 (Publication Type: Case Reports, English Abstract, Journal Article)

Chemical References

• Glucocorticoids
• Prednisolone

Topics

• Combined Modality Therapy
• Female
• Glucocorticoids (therapeutic use)
• Humans
• Middle Aged
• Myxedema (complications, drug therapy)
• Photopheresis
• Prednisolone (therapeutic use)
• Remission Induction
• Scleroderma, Systemic (complications, drug therapy)