Two Cases of Papular Mucinosis / 대한피부과학회지

ABSTRACT

Scleromyxedema is a rare fibromucinous disorder that is often difficult to treat and is associated with significant morbidity and mortality. Clinically, it shows white or flesh colored, dome-shaped waxy papules on the hands, arms, face, neck and upper trunk. There are proliferation of fibroblasts and mucin deposition in dermis and presence of serum paraproteins. It should be differentiated from scleroderma because of sclerotic skin changes. Patients were a 70 year old male and a 45 year old female. They showed multiple confluent or linearly arranged waxy papules on the head, neck and extremities. Sclerotic plaques were also seen. Abnormal findings were not detected in laboratory tests and bone marrow biopsy. Histologically, mucin deposition and marked proliferation of fibroblasts were seen in dermis. Therapy was performed with 200 mg/day of cyclophosphamide. Both patients showed good response and had no recurrence. The latter one had shown improvement of skin lesions but subsequently she developed leukocytopenia. Cyclophosphamide was stopped temporarily until the abnormal leukocytes count recovered. During therapy, mild hemiparesis developed. We could not find the cause of the symptom through physical examination and radiologic studies.