Scleromyxedema Myopathy: Histochemical and Electron Microscopic Observations

Verity, M. Anthony; Toop, James; McAdam, Lawrence P.; Pearson, Carl M.

doi:10.1093/ajcp/69.4.446

Cited by 33 publications

(14 citation statements)

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“…Verity et al . reported that this myopathy shared histopathological features with DM; 2 however, no photosensitivity, heliotrope erythema, erythema of the dorsum of the hands, Gottron's papules or antinuclear antibodies suggestive of DM have been described in association with this scleromyxedema‐associated myopathy, which is therefore probably a distinct entity. Fourthly, a real association between scleromyxedema and DM has only exceptionally been reported 6,7 .…”

Section: Discussionmentioning

confidence: 83%

“…However, this mucinosis is rarely clinically detectable; 13–15 it is typically a secondary mucinosis whose pathogenesis remains unclear but probably involves fibroblast stimulation by cytokines such as interleukin‐1 or interferon 16,17 . Thirdly, scleromyxedema can be associated with a myopathy that differs from DM 2–5 . Usually, scleromyxedema‐associated myopathy occurs months or even years after the onset of scleromyxedema but myopathy preceding scleromyxedema has also been reported.…”

Section: Discussionmentioning

confidence: 99%

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Scleromyxedema (lichen myxedematosus) associated with dermatomyositis

Launay¹,

Hatron²,

Delaporte

et al. 2001

Br J Dermatol

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A 41-year-old white man is described with papules of the lower and upper back, the neck and the upper chest, a marked deposition of mucin in the upper reticular dermis, and an IgG lambda monoclonal gammopathy strongly evocative of scleromyxedema (lichen myxedematosus). Additionally, he developed intense myalgia, muscle weakness and rhabdomyolysis, which were associated with heliotrope erythema, photosensitivity and an erythematous rash of the dorsum of the hands with Gottron's papules. Muscle biopsy revealed an inflammatory myositis, and dermatomyositis was diagnosed. The association of dermatomyositis and secondary mucinosis, or muscle involvement in primary papular mucinosis are not rare. However, the association between scleromyxedema and dermatomyositis has only exceptionally been reported.

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Section: Discussionmentioning

confidence: 83%

Section: Discussionmentioning

confidence: 99%

Scleromyxedema (lichen myxedematosus) associated with dermatomyositis

Launay¹,

Hatron²,

Delaporte

et al. 2001

Br J Dermatol

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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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“…Reported treatments include systemic retinoids, [17][18][19] corticosteroids (including dexamethasone 20 ), adrenocorticotropic hormone, 12 interferon), 22 2-chlorodeoxyadenosine, 23 granulocyte colony-stimulating factor, 24 thalidomide, 4 plasmapheresis, 25,26 dermabrasion, 27 electron beam radiation, 28 extracorporeal photochemotherapy, 29 and PUVA. 30 Other agents, including melphalan, 31-33 cyclophosphamide, 34 cyclosporine, 35-37 methotrexate, 38,39 and chlorambucil, 40 have been used with some success but are limited by toxicity. Prednisone has also been used successfully: Rayson et al 41 reported a case of a 40-year-old woman with scleromyxedema associated with a monoclonal IgG-lambda and with severe cutaneous and systemic manifestations.…”

Section: Discussionmentioning

confidence: 99%

Combination Oral Prednisone and Intravenous Immunoglobulin in the Treatment of Scleromyxedema

Majeski¹,

Taher²,

Grewal³

et al. 2005

J Cutan Med Surg

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IVIg is being used for a growing number of inflammatory and immune disorders. It is being increasingly reported as a successful treatment for scleromyxedema. Although our patient succumbed to the disease, combination therapy with prednisone and IVIg provided temporary symptomatic, laboratory, and clinical improvement of the condition. Optimization of this therapeutic strategy is thus indicated for the management of scleromyxedema.

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Scleromyxedema Myopathy: Histochemical and Electron Microscopic Observations

Cited by 33 publications

References 0 publications

Scleromyxedema (lichen myxedematosus) associated with dermatomyositis

Scleromyxedema (lichen myxedematosus) associated with dermatomyositis

Thalidomide as a Potential Treatment for Scleromyxedema

Combination Oral Prednisone and Intravenous Immunoglobulin in the Treatment of Scleromyxedema

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