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May 1972

Picture of the Month

Author Affiliations

Boston; Philadelphia
From St. Christopher's Hospital for Children, Philadelphia.

Am J Dis Child. 1972;123(5):485-487. doi:10.1001/archpedi.1972.02110110113012

Denouement and Discussion 

Focal Scleroderma 

Manifestations  Symptoms may start as early as 2 weeks of age. Skin lesions are present mainly on the face, trunk, and extremities and vary in size and shape. Involvement is usually unilateral but occasionally may be bilateral. When limited to the scalp or side of the face, the lesion is called a coup de sabre. First noted are localized erythema and edema. The lesions then become firm and waxy colored; occasionally there may be a surrounding violaceous halo. Later the lesions become atrophic and hypopigmented. Scarring may lead to secondary atrophy of the underlying muscles, contractures, demineralization, and alteration of local bone growth. On occasion there may be subcutaneous calcium deposits.Complete remissions are common and may last from months to many years following the onset. However, chronicity does occur and in one recorded case activity was still present after 33 years. Disability depends upon