Morphea

Morphea

Frontal linear scleroderma

Frontal linear scleroderma

Morphea

Morphea is a localized form of scleroderma characterized by patches of hardened skin that can appear anywhere on the body, including the face, hands, and feet. Unlike some other forms of scleroderma, morphea does not involve internal organs.

Signs and Symptoms

Morphea usually appears as macules or plaques that are a few centimeters in diameter. They can also present as bands, guttate lesions, or nodules. As a result of excessive collagen deposition, there's a thickening and hardening of the skin and subcutaneous tissues.

Variations

• Frontal linear scleroderma: This is a type of linear scleroderma characterized by a band of atrophy and a furrow in the skin. It often appears on the frontal or frontoparietal scalp and is sometimes referred to as "en coup de sabre" or "morphea en coup de sabre". Multiple lesions may coexist in a single patient, and some evidence suggests these lesions follow Blaschko's lines.
• Atrophoderma of Pasini and Pierini: This disease manifests as large lesions with sharp peripheral borders dropping into a depression. It results in round or oval patches of hyper-pigmented skin which may initially have a bluish or purplish hue.

Cause

The exact cause of morphea remains unknown. However, there's evidence suggesting a higher prevalence of autoimmune diseases in the families of morphea patients. Certain autoantibodies, such as anti-histone and anti-topoisomerase IIa, have been found in higher frequencies among morphea sufferers. Furthermore, cases of morphea coexisting with other systemic autoimmune diseases have been reported. Additionally, there are suggestions that Borrelia burgdorferi infection may play a role in the onset of a particular type of early-onset morphea, characterized by evident autoimmune phenomena.

Classification

Morphea can be classified into several types based on presentation:

• Morphea–lichen sclerosus et atrophicus overlap: This condition combines lesions of morphea with those of lichen sclerosus et atrophicus and is more prevalent in women.
• Pansclerotic morphea: This form involves sclerosis of multiple layers including the dermis, panniculus, fascia, and sometimes the bone. It can cause significant functional impairment due to the limitation of joint motion.
• Linear scleroderma: This autoimmune disease is identified by a line of thickened skin which can also affect underlying bones and muscles. Commonly seen in the arms, legs, or forehead, it predominantly affects one side of the body and usually first appears in young children.

Treatment

Various treatments have been explored for morphea:

• Corticosteroids: These can be applied topically, injected intra-lesionally, or taken systemically.
• Antimalarials: Drugs like hydroxychloroquine or chloroquine have been used.
• Immunomodulators: Medications such as methotrexate, topical tacrolimus, and penicillamine have been tried. In cases of active morphea in children and teenagers, the combination of oral methotrexate and prednisone has shown more promising results compared to placebo and prednisone alone.

Epidemiology

Morphea is more prevalent in women, with a ratio of approximately 3:1. It can manifest in childhood or during adult life. While relatively rare, it is estimated to affect 2 to 4 out of every 100,000 individuals.

See Also

• List of cutaneous conditions
• Frontal linear scleroderma (morphea en coup de sabre)
• scleroderma

Morphea Resources
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