Morphea, generalized

Morphea, Generalized

Morphea, also known as localized scleroderma, is a rare skin condition characterized by excessive collagen deposition leading to skin thickening and hardening. Generalized morphea is a subtype of morphea that involves multiple areas of the body, often symmetrically, and can lead to significant morbidity due to its extensive nature.

Pathophysiology

The pathogenesis of generalized morphea is not fully understood, but it is believed to involve an autoimmune process. The condition is characterized by an overproduction of collagen by fibroblasts, leading to thickening of the dermis and subcutaneous tissues. This process is thought to be triggered by a combination of genetic predisposition and environmental factors, such as infections or trauma.

Clinical Presentation

Patients with generalized morphea typically present with multiple, large, indurated plaques on the skin. These plaques are often hyperpigmented or hypopigmented and can be associated with itching or pain. Over time, the skin may become atrophic and lose its elasticity, leading to functional impairment, especially if joints are involved.

Diagnosis

The diagnosis of generalized morphea is primarily clinical, supported by histopathological examination of a skin biopsy. The biopsy typically shows thickening of the dermis with increased collagen deposition and a lymphocytic infiltrate. Laboratory tests may show elevated levels of antinuclear antibodies (ANA) or other autoantibodies, but these are not specific for morphea.

Treatment

Treatment of generalized morphea aims to halt disease progression and manage symptoms. Options include:

• Topical therapies: High-potency corticosteroids or calcineurin inhibitors can be used to reduce inflammation.
• Systemic therapies: Methotrexate and systemic corticosteroids are commonly used for more severe cases.
• Phototherapy: UVA1 phototherapy has been shown to be effective in some patients.

Prognosis

The course of generalized morphea is variable. Some patients experience spontaneous remission, while others may have persistent or progressive disease. Early intervention can help prevent complications and improve quality of life.

Also see

• Scleroderma
• Autoimmune diseases
• Collagen
• Dermatology