An Overview of Scleredema

Scleredema is a relatively rare skin disorder in which the skin thickens and hardens, sometimes with a red discoloration. What causes scleredema is not known. It is often associated with diabetes, and among people with diabetes, men are affected much more often than women (10:1).

Scleredema may occur after a viral illness or streptococcal throat infection, and in these cases, females are affected twice as often as males. Despite being sometimes called scleredema adultorum, the disorder occurs in individuals of all ages and all ethnic backgrounds.

Symptoms

When scleredema occurs, the affected area of skin becomes thickened and hardened. It may become red or darkened in color, and may look rough like the skin of an orange (called peau d’orange). In most cases, scleredema begins on the face, neck, or upper back. It may spread to the arms or chest, but the hands and feet are not usually affected. Other symptoms may occur depending on the area affected:

• Face: Difficulty in opening the eyes or mouth
• Skin over joints: Limited motion
• Tongue: Difficulty speaking or chewing

In some individuals, scleredema affects not just skin but other body parts such as the heart, liver, spleen, muscles, or throat.

Diagnosis

Scleredema is usually suspected based on the appearance of the skin and the medical history of the individual (such as having diabetes or a recent infection). A skin sample (biopsy) is taken and examined under the microscope to confirm the diagnosis.

Other tests may be performed, such as a throat culture to look for streptococcal throat infection and blood glucose to test for diabetes. Another special blood test is sometimes done to check for a blood disease such as multiple myeloma, which may appear after scleredema begins.

Treatment

Many different types of medications have been tried as treatments for scleredema, but no one medication has been proven to be best. Individuals may benefit from corticosteroids, cyclosporine, methotrexate, UVA1 phototherapy, or psoralen with ultraviolet light A. If an infection is present, antibiotics may be required. If diabetes is present, it should be controlled with diet, exercise, and medication. Physical therapy may help if any movement of body parts is restricted.

Individuals who develop scleredema after infection tend to have a shorter course of the disease, with symptoms resolving within 6 months to 2 years. Individuals with diabetes who develop scleredema have symptoms for a longer period of time, often many years.