Lich myxedematosus. A dermal infiltrative disorder responsive to cyclophosphamide therapy

Howsden, Susan M.

doi:10.1001/archderm.111.10.1325

Cited by 39 publications

(18 citation statements)

References 21 publications

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“…7,17,18 Other reports of successful treatment include granulocyte colonystimulating factor, 19 plasmapheresis, 20 external beam irradiation, 21 intravenous immunoglobulin, 22 interferon alfa2a, 23 and extracorporal photopheresis. 24 Multiple chemotherapeutic agents have been used, including melphalan, 5,25,26 cyclosporine, 27 cladribine, 28 cyclophosphamide, 29,30 methotrexate, 31,32 and chlorambucil. 33 Despite all these reported trials, most patients with scleromyxedema do not show a significant response to therapy.…”

Section: Commentmentioning

confidence: 99%

Thalidomide as a Potential Treatment for Scleromyxedema

Caradonna¹

2004

Arch Dermatol

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with a 1-year history of skin thickening. The process began on her hands and progressed to involve her arms, face, neck, shoulders, chest, and proximal extremities. Her only systemic complaints were limited mobility of her joints, particularly her hands and wrists. Physical examination findings revealed leonine facies. The skin of the neck, shoulders, and chest exhibited 1-to 2-mm coalescent linear papules. The skin of the hands, forearms, upper arms, and trunk was sclerotic. Skin biopsy results showed marked dermal thickening, displacement of collagen bundles by mucin, and numerous spindled fibroblasts, consistent with scleromyxedema. The results of laboratory tests, including a complete blood cell count, chemistry tests, liver function tests, an antinuclear antibody panel, an extractable nuclear antigen panel, and thyroid function tests, were normal. Immunoglobulins were obtained, and the following levels were revealed: IgM, 92 mg/dL (reference range, 25-210 mg/ dL); IgA, 152 mg/dL (reference range, 40-390 mg/dL); and IgG, 1490 mg/dL (reference range, 525-1650 mg/ dL), with a light chain monoclonal gammopathy. Hematologic evaluations, including serum and urine protein electrophoresis, immunoelectrophoresis, a bone marrow examination, and radiography of the head and chest, produced no evidence of multiple myeloma.Because of significant restriction of joint mobility, the patient underwent treatment with cytotoxic agents. She was initially treated with melphalan, 16 mg/d orally (PO), plus prednisone, 100 mg/d PO, for 4 days at a time in 1-month intervals for 4 cycles from

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Section: Commentmentioning

confidence: 99%

Thalidomide as a Potential Treatment for Scleromyxedema

Caradonna¹

2004

Arch Dermatol

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“…To date the only forms of treatment which have had a beneficial effect are radiotherapy [5][6][7] and cytostatic drugs, particularly melphalan [8][9][10]. cyclo phosphamide [11]. vincristine [12] and methotrexate [13].…”

Section: Introductionmentioning

confidence: 99%

Normalization of Skin Appearance in a Patient with Scleromyxoedema after Intensive Chemotherapy for Hodgkin’s Disease

Alberts¹,

Schulz

Falkson³

et al. 1989

Dermatology

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A patient with scleromyxoedema was treated for 6 years with cytostatic drugs. During this time the skin lesions followed a fluctuating but progressive course. After 6 years she developed Hodgkin’s lymphoma of the mixed cellularity type. Intensive cytostatic treatment given for Hodkin’s disease resulted in virtually complete disappearance of the scleromyxoedema lesions. The development of Hodgkin’s disease is considered fortuitous and not due to the previous cytostatic drugs.

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“…The first type is the most common and is also called sclero Dermatology 1996:192:268 myxedema. Although the clinical course of LM may be benign, most patients have a plasma cell dyscrasia, as evidenced by a se rum monoclonal paraprotein [10][11][12], How ever, there are also some reports in which a paraprotein was not present [13][14][15]. Para proteinemia has not been found in any cases of SJCM |2 -6 |.…”

Section: Discussionmentioning

confidence: 99%

Self Healing Cutaneous Mucinosis

et al. 1996

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Self-healing juvenile cutaneous mucinosis is a new entity distinct from previously described forms of cutaneous mucinosis. We present a new case in a 26-year-old female patient who began abruptly with a papular eruption, involving the scalp, face, neck and trunk, accompanied by periarticular papules on the hands and arthralgias. No associated disease could be detected and the lesions resolved spontaneously within a few months. This is the first case described in an adult patient.

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Lich myxedematosus. A dermal infiltrative disorder responsive to cyclophosphamide therapy

Cited by 39 publications

References 21 publications

Thalidomide as a Potential Treatment for Scleromyxedema

Thalidomide as a Potential Treatment for Scleromyxedema

Normalization of Skin Appearance in a Patient with Scleromyxoedema after Intensive Chemotherapy for Hodgkin’s Disease

Self Healing Cutaneous Mucinosis

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