1/118. scleromyxedema: treatment with interferon alfa. scleromyxedema is a variant of papular mucinosis characterized by fibroblast proliferation and mucin deposition in the dermis. Historically, it has been very difficult to treat and can cause significant morbidity and mortality with systemic involvement. We describe a case of a woman with scleromyxedema and systemic manifestations treated with interferon alfa. Her skin responded very well to therapy within 3 months; however, her systemic manifestations showed little change. We conclude that interferon alfa may be a useful therapy for patients with scleromyxedema, particularly if the disease process is limited to the skin. ( info) |
2/118. Treatment of reticular erythematous mucinosis with a large dose of ultraviolet B radiation and steroid impregnated tape. To date, there are no successful treatments for reticular erythematous mucinosis (REM) other than antimalarial drugs such as chloroquine; these have many adverse side effects and are prohibited for use in japan. We report a case of REM improved by a large dose of ultraviolet B (UVB) radiation and a steroid impregnated tape. The large dose of UVB radiation improved the erythema after 5 weeks. Application of steroid impregnated tape to a lesion where a large dose of UVB had been given produced an additive clinical effect. UVB radiation and steroid impregnated tape are considered to be effective treatments for REM. ( info) |
3/118. Dermal mucinosis and musculoskeletal symptoms simulating polymyositis as a presenting sign of hypothyroidism. A case of dermal mucinosis and musculoskeletal symptoms simulating polymyositis as a presenting sign of hypothyroidism is presented. The patient presented muscle weakness and edema of the face including the eyelids. Laboratory examination revealed elevated creatinine phosphokinase, decreased free-T4, decreased free-T3, elevated TSH, positive anti-microsome antibody and positive anti-TSH receptor antibody. The skin biopsy specimen revealed swelling of the collagen bundles with the bundles splitting up into individual fibers, with some blue threads and granules of mucin interspersed. Alucian blue stain demonstrated vast amounts of mucin throughout the whole dermis, which was completely removed on incubation with streptomyces hyaluronidase. The patient was diagnosed as having hypothyroidism due to Hashimoto's disease with possible polymyositis complications. After two months of thyroid hormone replacement therapy, she was euthyroidic and discharged. These results indicate that our case was a rare case of severe generalized myxoedema due to hypothyroidism of Hashimoto's disease simulating the musculoskeletal symptoms of polymyositis. ( info) |
| scleromyxedema is a disorder characterized by a typical rash due to the accumulation of mucin in the dermis. It is always associated with a monoclonal protein in the serum and can have a wide variety of systemic manifestations. We describe a 40-year-old woman who had scleromyxedema associated with a monoclonal G lambda protein. Severe systemic symptoms included fatigue, esophageal dysmotility, and myopathy. Symptoms resolved completely with oral prednisone therapy, and she remained in clinical remission 24 months after use of prednisone was discontinued. scleromyxedema is commonly treated with alkylating agents, which have been associated with pronounced morbidity and mortality. We suggest that oral corticosteroid therapy may be a reasonable initial choice for treating this disease and that alkylating agents be reserved for corticosteroid-refractory disease. ( info) |
5/118. Cutaneous mucinous nodules associated with proliferating synovitis of rheumatoid arthritis. Rheumatoid arthritis (RA) is occasionally associated with specific or non-specific cutaneous conditions. In this report, we describe unusual cutaneous nodules arising on the skin overlying the inflamed joints of two patients with RA. The nodules were histopathologically characterized by mucinous granulation tissue associated with neutrophilic infiltration and proliferation of starry-shaped mesenchymal cells. The affected joints of both cases showed synovitis with synovial tissue hyperplasia due to RA. Because hyaluronic mucin, which is the major component of synovial fluids, was deposited in the nodules, intradermal inoculation of synovial cells seemed to be responsible for the nodular development. The nodules of one patient spontaneously resolved in several months. We are unaware of any report describing similar cases. Such nodules should be included as a cutaneous complication of RA, distinct from neoplasias. ( info) |
| scleromyxedema is a sclerotic variant of papular mucinosis, in which lichenoid papules and scleroderma-like features are present. We describe a patient with scleromyxedema with IgG type lambda chain paraprotein, a systemic sclerosis-like illness, and myositis. The patient's serum contained Scl 70 antibodies, characteristic of scleroderma. electromyography showed signs of acute myositis and the creatine phosphokinase (CPK) level was elevated. Multiply passaged fibroblasts from the patient's skin lesions showed altered growth response in vitro. The patient was treated with cyclosporin (4 mg/kg/day) with improvement. ( info) |
7/118. Self-healing juvenile cutaneous mucinosis. Self-healing juvenile cutaneous mucinosis was encountered in a 15-year-old boy who presented with characteristic cutaneous findings and a clinical course of final spontaneous resolution. The interesting point is the presence of numerous nodules found in unusual locations. To date, nodules occurring on the long axis of the extremities have not been reported. ( info) |
| The authors monitored CSF findings for over 5 months in a patient with a fatal case of scleromyxedema and two episodes of encephalopathy. During both encephalopathy episodes, CSF protein and immunoglobulin g (IgG) levels were elevated without an increased IgG index or IgG synthesis rate. A CSF-dominant increase in the concentration of interleukin-6 (IL-6) also was noted during encephalopathy. These findings suggest a disruption of the blood-brain barrier and that IL-6 may play some role in mediating the encephalopathy. OFF ( info) |
9/118. scleromyxedema is a scleroderma-like disorder and not a coexistance of scleroderma with papular mucinosis. We present four cases of scleromyxedema with scleroderma-like cutaneous changes mimicking systemic sclerosis and stress the importance of their differentiation from true scleroderma. scleromyxedema should be recognized as an entity since it differs from scleroderma in the pathogenesis, histopathology of cutaneous lesions, type of visceral involvement (if present), frequent association with paraproteinemia, the course and prognosis. ( info) |
10/118. Acute periorbital mucinosis in discoid lupus erythematosus. Periorbital edema associated with lupus erythematosus is not frequently reported. To our knowledge, periorbital edema from increased dermal mucin has not been reported with any form of lupus. We present a patient with discoid lupus exhibiting periorbital edema from massive mucinosis. ( info) |