Original articleScleromyxedema: A multicenter study of characteristics, comorbidities, course, and therapy in 30 patients
Section snippets
Methods
All patients seen from January 1, 2000, to April 1, 2012, and given a diagnosis of scleromyxedema at the participating centers were evaluated retrospectively by chart review and prospectively. The diagnosis of scleromyxedema was made on the basis of the following criteria: (1) a generalized papular and sclerodermoid eruption; (2) microscopic evidence of mucin deposition, fibroblast proliferation, and fibrosis; (3) detection of monoclonal gammopathy; and (4) absence of a concomitant thyroid
Results
A total of 30 patients meeting our clinicopathologic criteria for scleromyxedema were included in this study. In all, 27 patients fulfilled all 4 criteria for diagnosing scleromyxedema and 3 were classified as atypical because they lacked monoclonal gammopathy. There were 17 men and 13 women. The mean age was 59 years (range, 34-86). The mean duration of the skin disease at diagnosis was 9 months (range, 1-60). History was not contributory for most of them, except for 4 patients who presented
Discussion
Most information about scleromyxedema has been retrieved from anecdotal or single case reports. The largest series on scleromyxedema comparable with ours, published in 1995,3 included 26 patients. This series was an update to the Mayo Clinic medical records including a number of patients who had been previously reported. Only small collections of published cases are further available for comparison including another retrospective series of 10 patients from the Johns Hopkins University4 and a
References (28)
- et al.
Updated classification of papular mucinosis, lichen myxedematosus, and scleromyxedema
J Am Acad Dermatol
(2001) - et al.
Scleromyxedema
J Am Acad Dermatol
(1995) - et al.
Clinical characteristics, outcome of scleromyxedema: a retrospective multicenter study
Ann Dermatol Venereol
(2010) - et al.
High-dose dexamethasone in scleromyxedema: report of 2 additional cases
J Am Acad Dermatol
(2005) - et al.
Treatment of recalcitrant scleromyxedema with thalidomide in 3 patients
J Am Acad Dermatol
(2004) - et al.
Scleromyxedema: role of high-dose melphalan with autologous stem cell transplantation
Blood
(2006) - et al.
Treatment of scleromyxedema and the dermatoneuro syndrome with intravenous immunoglobulin
J Am Acad Dermatol
(2009) - et al.
Scleromyxedema with dermato-neuro syndrome
J Am Acad Dermatol
(2000) - et al.
Fatal scleromyxedema: report of a case and review of the literature
J Am Acad Dermatol
(1998) - et al.
Lichen myxedematosus with systemic involvement: clinical and autopsy findings
J Am Acad Dermatol
(2001)
Scleromyxedema
Clin Dermatol
Mucinoses
Scleromyxedema: a case series highlighting long-term outcomes of treatment with intravenous immunoglobulin (IVIG)
Medicine (Baltimore)
Scleromyxedema with an interstitial granulomatous-like pattern: a rare histologic variant mimicking granuloma annulare
J Cutan Pathol
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Funding sources: None.
Conflicts of interest: None declared.