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Disabling Pansclerotic Morphea of Childhood Poses a High Risk of Chronic Ulceration of the Skin and Squamous Cell Carcinoma

Department of Dermatology and Allergology, wollina-uw{at}khdf.de

Michael Buslau

Kantonsspital Schaffhausen, Switzerland

Birgit Heinig

Department of Physical Therapy

Ivailo Petrov

Department of Dermatology, Medical University of Sofia, Sofia, Bulgaria

Eleonore Unger

Department of Internal Medicine I/Rheumatology

Eugenia Kyriopoulou

Department of Dermatology, Medical University of Sofia, Sofia, Bulgaria

André Koch

Department of Dermatology and Allergology

Erich Köstler

Department of Dermatology and Allergology

Jaqueline Schönlebe

Institute of Pathology "Georg Schmorl," Hospital Dresden-Friedrichstadt, Dresden, Germany

Gunther Haroske

Institute of Pathology "Georg Schmorl," Hospital Dresden-Friedrichstadt, Dresden, Germany

Thorsten Doede

Department of Pediatric Surgery, Medical School-Freidrich Schiller University of Jena, Jena, Germany

Kyrill Pramatarov

Department of Dermatology, Medical University of Sofia, Sofia, Bulgaria

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.

Key Words: deep morphoea • disabling pansclerotic morphoea of childhood • chronic (leg) ulcers • squamous cell carcinoma • sildenafil • tissue-engineered skin substitutes

The International Journal of Lower Extremity Wounds, Vol. 6, No. 4, 291-298 (2007)
DOI: 10.1177/1534734607308731


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