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Disabling pansclerotic morphea of childhood (DPMC) is a rare and severe variant of scleroderma. This report presents 3 cases that presented to the authors and studies 25 patients from the literature (English language only) for the presence of chronic nonhealing ulcers of skin and skin cancer. The authors identified a total of 30 patients (9 male and 21 female) aged between 1 and 37 years at time of presentation. All cases were less than 14 years old when the disease started. The majority of patients had an aggressive course with deep sclerotic lesions leading to joint contractures and immobility. Five patients suffered from chronic nonhealing leg ulcers (17%), but ulcers were present on other parts of the body (upper limbs, trunk, head) as well (n = 6). Four patients died because of complications of the disease such as sepsis or gangrene. Two patients developed a squamous cell carcinoma at the age of 16 years and 19 years, respectively (6.7%). The available treatment of DMPC-associated ulcers is unsatisfying. Only temporary improvements have been seen in a minority of patients. We report on marked improvement of chronic leg ulcers by a combination of sildenafil 3 x 20 mg/day and repeated application of a porcine small intestinal submucosal acellular matrix.

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SUMMARY Cutis marmorata telangiectatica congenita was observed in two adult sisters. The condition appeared at birth and did not change much with age. In one sister the condition was accompanied by hypertension, acrocyanosis and ulceration of the big toe. On the basis of a comparison of the symptoms in patients and their relatives, the authors assume that Van Lohuizen syndrome is a dominantly inherited genetic disorder with low penetrance and great intrafamilial variability.

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