Scleredema

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What is scleredema?

Scleredema is characterized by firm, nonpitting skin edema (“woody”) that typically begins on the neck and upper back and spreads to the shoulders and trunk. The face and extremities (typically upper) can also be involved. The hands and feet are spared. The onset of skin involvement can be abrupt. Skin biopsy typically shows normal epidermis but thickened dermis with broad bands of collagen that appear separated on histology due to mucin deposition between the collagen bundles. There are three subtypes of scleredema based on the underlying cause:

• Type I: scleredema adultorum (scleredema of Buschke) and scleredema neonatorum follow an infection or febrile illness, with resolution occurring in months to years. Female predominance (2:1) with age of onset commonly <20 years.

• Type II: scleredema with no preceding febrile illness and a slow, progressive course. There is a female predominance (2:1). Frequently associated with paraproteinemia (most commonly IgG, IgA). Progression to multiple myeloma has been reported in 25% to 45% of patients.

• Type III: scleredema diabeticorum occurs in association with poorly controlled diabetes mellitus (type I or II). There is a male predominance (10:1), and prevalence of 2% to 10% has been reported among patients with diabetes.

Although there is no consistently effective therapy, Type I scleredema may be mild and self-limiting and therefore treatment may not be necessary. In patients with protracted courses with symptomatic lesions and/or functional impairment, ultraviolet A1 (UVA1) phototherapy has been effective in case reports and represents the first-line therapy. Additional agents that have been tried in scleredema with variable success include cyclosporine, methotrexate, and tamoxifen.

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