Scleredema Workup

Updated: Mar 15, 2019
  • Author: Lisa K Pappas-Taffer, MD; Chief Editor: Dirk M Elston, MD  more...
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Workup

Laboratory Studies

Laboratory studies can be helpful to identify an associated condition:

A throat culture should be obtained in scleredema patients to exclude group A streptococcal infection.

Antistreptolysin-O (ASO) titers should be obtained in scleredema patients to exclude a recent infection with group A streptococci in a patient without clinically apparent pharyngitis or other infectious etiology.

Fasting blood glucose or glycosylated hemoglobin (A1C) measurements should be obtained in scleredema patients to rule out diabetes mellitus.

Serum protein electrophoresis (SPEP) and immunofixation should be performed in scleredema patients to exclude a monoclonal gammopathy. Both paraproteinemias (also known as monoclonal gammopathies) and multiple myelomas have been reported in patients with scleredema (type 2). The most commonly identified paraprotein is IgG having a kappa light chain; however, an IgA type can occur. Blood dyscrasias usually appear several years after the onset of scleredema. The interval between diagnosis of scleredema and the detection of paraprotein can occur before, concurrently, or after the diagnosis of scleredema. In one report, a delayed paraproteinemia was detected on average up to 10 years following the diagnosis of scleredema. [13] However, other sources quote a median detection of paraproteinemia 2.5 years after diagnosis.

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Imaging Studies

No imaging studies are necessary for the diagnosis of scleredema.

Isolated case reports have described scleredema occurring in association with internal malignancies (eg, carcinoma of the gall bladder, [5] malignant insulinoma, [30] carcinoid tumor [6] ). Imaging studies are warranted if this is suggested based on clinical findings.

Some investigators have proposed ultrasonic imaging of skin thickness in scleredema patients as a method to monitor the response to therapy. Computed tomography may demonstrate induration in the involved soft tissues, although it is not often necessary. MRI may highlight the involved areas as well, and some have advocated for MRI usage to determine the extent of disease and monitor progression in select cases. [31]

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Other Tests

A punch biopsy or incisional biopsy of lesional skin that extends to include subcutaneous fat is recommended.

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Histologic Findings

The histopathologic analysis reveals a normal epidermis with a thickened dermis and increased spaces between large collagen bundles. The space results from increased deposition of mucopolysaccharide (hyaluronic acid) in the dermis. The mucin is more prominent in the deep dermis. In some cases, mucin is better detected in unfixed sections stained at a pH of 7.0 with toluidine blue, or in tissue fixed with 0.05% or 1% cetylpyridinium chloride solution and stained with Alcian blue at a pH of 2.5.

Appendiceal structures in scleredema remain unchanged (unlike scleroderma) and there is an absence of a fibroblast proliferation (unlike scleromyxedema or nephrogenic systemic fibrosis).

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