Sclerodema

Sclerodema is a rare connective tissue disorder characterized by hardening and thickening of the skin. It typically affects adults, with a higher prevalence in women. The exact cause of sclerodema is unknown, but it is thought to be an autoimmune condition. It can occur spontaneously or be associated with other diseases, including diabetes mellitus, infections, and certain cancers.

The skin in sclerodema becomes firm, indurated, and often waxy. It can be difficult to pinch the affected skin, and movement may become restricted due to tightness. The face, neck, shoulders, and upper back are commonly involved, though the condition can spread to other areas of the body. While the skin changes are the primary feature, some individuals may experience joint pain or stiffness. Sclerodema is generally not painful, but the immobility caused by skin thickening can impact quality of life.

Diagnosis is usually made based on clinical examination and skin biopsy, which shows characteristic changes in the dermis. Treatment aims to manage symptoms and prevent progression. Options may include physical therapy, topical or oral corticosteroids, and other immunosuppressive medications. The prognosis for sclerodema varies, with some individuals experiencing stabilization while others may have progressive disease.