Extracorporeal photochemotherapy: A potentially useful treatment for scleromyxedema

Berkson, Michael; Lazarus, Gerald S.; Uberti-Benz, Marie; Rook, Alain H.

doi:10.1016/s0190-9622(08)80676-7

Cited by 52 publications

(28 citation statements)

References 4 publications

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“…5 The paraproteinemia differs from normal IgG because it is extremely basic globulin of a small size (molecular mass: 110Kda; normal: 160Kda) 8 and because of the absence of the significant antigen portion of the Fd fragment. 5,9 In the reported case, the lambda IgG type monoclonal paraproteinemia was observed.…”

Section: Discussionmentioning

confidence: 99%

“…1,5 As a response to the indiscriminate use of the terms papular mucinosis, myxedematous lichen, and scleromyxedema, in 2001, Rongioletti et al, 6 based on anatomic-clinical criteria, adopted a new classification for this group of diseases: (1) generalized myxedematous lichen (scleromyxedema); (2) localized myxedematous lichen; (3) atypical forms of myxedematous lichen.…”

Section: Introductionmentioning

confidence: 99%

“…Cosmetically, dermal abrasion 5 and CO 2 laser, 10 have been used, but the treatment does not prevent the appearance of new lesions.…”

mentioning

confidence: 99%

“…Other therapy modalities that have been reported, with inconsistent results, include conventional radiation therapy, 5 chemotherapy agents, 7 extracorporeal photochemotherapy, 5 isotretinoin, talidomide, plasmapheresis, 13 Puva, 5 cloroquin 3 and IV immunoglobulin.…”

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confidence: 99%

“…3,5,12 Because strogiloidiasis is an intestinal parasitosis frequently found in tropical and subtropical regions and its dissemination is related to the use of high doses of steroids for long periods16 prophylatic treatment is recommended, independently from the results of feces tests.…”

mentioning

confidence: 99%

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Escleromixedema: um caso tratado com prednisona oral

Neto

Sales

Silva

et al. 2006

An. Bras. Dermatol.

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Scleromyxedema is an idiopathic cutaneous mucinosis characterized by a papular eruption, skin induration and paraproteinemia. Histologically, fibrolast proliferation can be observed in the upper dermis associated with a mucine deposition. Treatment is difficult and at present there is no totally effective therapeutic modality to control the disease. The present report is on a 68-year-old patient with scleromyxedema without systemic manifestation, who responded to oral steroid therapy.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

“…Cosmetically, dermal abrasion 5 and CO 2 laser, 10 have been used, but the treatment does not prevent the appearance of new lesions.…”

mentioning

confidence: 99%

mentioning

confidence: 99%

mentioning

confidence: 99%

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Escleromixedema: um caso tratado com prednisona oral

Neto

Sales

Silva

et al. 2006

An. Bras. Dermatol.

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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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