Scleredema

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Although patients with this disorder develop induration (hardening) of the skin, scle-redema should not be confused with the distinctly different disease, scleroderma. The

From: Contemporary Endocrinology: Type 1 Diabetes: Etiology and Treatment Edited by: M. A. Sperling © Humana Press Inc., Totowa, NJ

Scleredema Type
Fig. 1. Peau d'orange appearance of the upper back in a patient with scleroderma. The areas of involvement are firm to palpation.

basic abnormality in scleredema is an increased deposition of glycosaminoglycans (mucin)—in particular, hyaluronic acid—within the dermis. The upper back is the most common site of involvement, followed by the neck, upper arms, and upper chest. Occasionally, there is facial involvement and, rarely, cardiac or esophageal involvement. Given the often subtle changes in the appearance of the skin, the diagnosis may be missed unless the skin is palpated. Then, the induration of the skin becomes obvious.

Visual clues to the diagnosis include prominent openings of the hair follicles and diffuse erythema. The former can give the skin an appearance that resembles the peel of an orange, hence the term peau d'orange (see Fig. 1). The diffuse erythema is most commonly observed on the upper back and, on occasion, it is misdiagnosed as treatment-resistant cellulitis. Although patients may notice a tightening of the skin, more frequently they comment on decreased mobility of the neck. The onset, however, is usually insidious. By physical examination, there is decreased range of motion, especially dorsal extension of the neck.

The diagnosis of scleredema is based on the above-outlined clinical features as well as the histologic finding of increased deposits of mucin within the dermis. The disorder is then further subdivided based on the presence or absence of diabetes mellitus. In 1 study of 33 patients with scleredema, those with diabetes usually had the late-onset, but insulin-requiring, form and were difficult to control (1). Of the group of patients without diabetes, some will be found to have a monoclonal gammopathy (2) or a preceding streptococcal infection.

An improvement of scleredema has been reported following better diabetic control via insulin, intravenous administration of prostaglandin E1, high-dose penicillin, psoralens plus ultraviolet A (PUVA), electron beam therapy, intralesional corticosteroids, and oral tocopherol acetate plus hyaluronidase (3-5). However, these therapeutic interventions are based primarily on case reports and small series of patients enrolled in open clinical trials. Based on personal experience, our initial therapeutic intervention in symptomatic patients

Table 1

Prevalence of Limited Joint Mobility (LJM) in Children 7-18 yr Old with Type 1 Diabetes and Percent of Those Children with Mild or Moderate/Severe LJM

Table 1

Prevalence of Limited Joint Mobility (LJM) in Children 7-18 yr Old with Type 1 Diabetes and Percent of Those Children with Mild or Moderate/Severe LJM

Year

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