Tumorous variant of scleromyxedema. Successful therapy with intravenous immunoglobulins

• Published in: Journal of the European Academy of Dermatology and Venereology Vol. 19; no. 4; pp. 462 - 465
• Main Authors: Wojas‐Pelc, A, Błaszczyk, M, Glińska, M, Jabłońska, S
• Format: Journal Article
• Language: English
• Published: Oxford, UK Blackwell Science Ltd 01.07.2005
• Subjects: Diagnosis, Differential; Facial Dermatoses - diagnosis; Facial Dermatoses - drug therapy; Facial Dermatoses - pathology; facies leonina; Female; high‐dose intravenous immunoglobulin; Humans; Immunoglobulins, Intravenous - administration & dosage; lymphoma‐like lesions; Middle Aged; Myxedema - diagnosis; Myxedema - drug therapy; Myxedema - pathology; scleromyxedema
• ISSN: 0926-9959; 1468-3083
• DOI: 10.1111/j.1468-3083.2005.01134.x

ABSTRACT We present an unusual tumorous variety of scleromyxedema mimicking facies leonina in lymphoma. In spite of pronounced and widespread cutaneous changes, hypergammaglobulinaemia and paraproteinaemia, the general condition of the patient was satisfactory, there was no internal involvement and no symptoms of any malignancy. Initially, melphalan and corticosteroids were applied but were not effective. High‐dose intravenous immunoglobulin (IVIG) therapy had dramatic effect, and after five 5‐day monthly courses the tumours almost regressed and the skin became less hard. After a further five courses in the following year there was complete clearance, which was sustained without any therapy for 1 year (until now). IVIG appears to be the therapy of choice for scleromyxedema. We stress, however, that at the start of therapy, IVIG applications should be supplemented with small doses of melphalan and/or corticosteroids.