Original article
Scleromyxedema: A multicenter study of characteristics, comorbidities, course, and therapy in 30 patients

https://doi.org/10.1016/j.jaad.2013.01.007Get rights and content

Background

Scleromyxedema is associated with a monoclonal gammopathy and other comorbidities. Its prognostic and therapeutic features are poorly documented because most reports deal with single cases or small series.

Objective

We sought to describe the characteristics of patients with scleromyxedema regarding demographics, clinical characteristics, comorbidities, therapeutic interventions, and course.

Methods

We conducted a retrospective and prospective multicenter study.

Results

We identified 30 patients with scleromyxedema (17 men and 13 women). The mean age at diagnosis was 59 years. The mean delay between disease onset and diagnosis was 9 months. Monoclonal gammopathy was detected in 27 patients. Extracutaneous manifestations were present in 19 patients including neurologic (30%), rheumatologic (23.3%), and cardiac (20%) manifestations. Two patients developed hematologic malignancies. The most common therapies included oral steroids and intravenous immunoglobulins. Although corticosteroids were ineffective, intravenous immunoglobulins (alone or in combination with other drugs) induced complete remission in 4 and partial remission in 9 patients with a mean treatment duration of 2 years. In all, 21 patients were followed up for a mean period of 33.5 months, at which time 16 patients were alive, 12 with and 4 without skin disease. Five patients died: 2 with dermatoneuro syndrome and 1 each with myeloid leukemia, Hodgkin lymphoma, and myocardial insufficiency.

Limitations

This is mainly a retrospective study.

Conclusions

Our study confirms that scleromyxedema is a chronic and unpredictable disease with severe systemic manifestations leading to a guarded prognosis. There is no specific definitive treatment. Our data support the contention that intravenous immunoglobulin is a relatively effective and safe treatment. The response is not permanent and maintenance infusions are required.

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

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    Funding sources: None.

    Conflicts of interest: None declared.

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