A diffuse rash in a man with paraproteinemia

CASE #1 Scleromyxedema Scleromyxedema is the generalized form of lichen myxedematosus, which presents with widespread eruption of shiny, firm, dome-shaped or flattopped papules; these papules do not affect the palms, scalp, or mucous membranes.1,2 Other clinical findings can include the doughnut sig...

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Bibliographic Details
Published in:The clinical advisor 2017-05, Vol.20 (5), p.49
Main Authors: Wallace, Matthew M, Ortega-Loayza, Alex G
Format: Magazinearticle
Language:English
Subjects:
Cardiomyopathy
Dysphagia
Dyspnea
Immunoglobulins
Lymphoma
Scleroderma
Skin
Stem cells
Thyroid gland
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Summary:CASE #1 Scleromyxedema Scleromyxedema is the generalized form of lichen myxedematosus, which presents with widespread eruption of shiny, firm, dome-shaped or flattopped papules; these papules do not affect the palms, scalp, or mucous membranes.1,2 Other clinical findings can include the doughnut sign, characterized by skin thickening with a central depression overlying the proximal interphalangeal joints, and decreased range of motion of the hands, lips, mouth, and extremities.1,2 There is no sex predilection in scleromyxedema.3 Mean age at onset of the condition is typically the fifth decade of life, but onset can present between the second and seventh decades.4,5 On average, the condition is diagnosed 3 years after initial onset.5 The localized form of lichen myxedematosus is papular mucinosis.1 Although papular mucinosis is not associated with extracutaneous findings, scleromyxedema has systemic manifestations in up to 77% of patients; in some cases, these manifestations are fatal.2,5 In a retrospective study of 26 patients with scleromyxedema, gastrointestinal involvement was the most common finding in 60% of patients-specifically, dysphagia due to esophageal dysmotility.2,5 In another retrospective study of 30 patients with scleromyxedema, other extracutaneous findings included neurologic abnormalities in 30% (eg, carpal tunnel syndrome and dermatoneuro syndrome [fever, seizures, and coma]), rheumatologic abnormalities in...
ISSN:1524-7317