Deep morphea

Abstract

Deep morphea encompasses a variety of clinical entities in which inflammation and sclerosis are found in the deep dermis, panniculus, fascia, or superficial muscle. Morphea profunda, eosinophilic fasciitis, and disabling pansclerotic morphea of children are included in this group, but overlapping of the extent and depth of cutaneous involvement in these various conditions precludes their distinction on the sole basis of clinical or even histologic examination. Furthermore, the limits between morphea profunda and generalized morphea, which usually are classified outside this group, are not clear. Histologically, all these disorders show similar inflammatory and sclerotic findings, the primary difference being the depth of these changes. Associated clinical findings, including arthralgias, arthritis, contractures, or carpal tunnel syndrome, are frequent. Although visceral complications are uncommon, pulmonary, esophageal, and even cardiac abnormalities have been reported. Eosinophilia, hypergammaglobulinemia, and increased erythrocyte sedimentation rate may be present with disease activity. Laboratory studies may demonstrate autoantibody production. Treatment is nonstandardized but UVA irradiation and antiinflammatory or immunosuppressive drugs (mainly antimalarial agents and corticosteroids) may be beneficial.