@article{3132543,
    title = "Disabling pansclerotic morphea of childhood poses a high risk of chronic ulceration of the skin and squamous cell carcinoma",
    author = "Wollina, U. and Buslau, M. and Heinig, B. and Petrov, I. and Unger, E. and Kyriopoulou, E. and Koch, A. and Köstler, E. and Schönlebe, J. and Haroske, G. and Doede, T. and Pramatarov, K.",
    journal = "International Journal of Lower Extremity Wounds",
    year = "2007",
    volume = "6",
    number = "4",
    pages = "291-298",
    issn = "1534-7346, 1552-6941",
    doi = "10.1177/1534734607308731",
    keywords = "antiinfective agent;  chlorhexidine;  cyclosporin A;  gentamicin;  immunoglobulin;  immunosuppressive agent;  methotrexate;  methoxsalen;  penicillamine;  penicillin G;  pentoxifylline;  prednisolone;  sildenafil;  steroid, absence of side effects;  adolescent;  adult;  anamnesis;  article;  child;  clinical article;  clinical feature;  compression therapy;  computer assisted tomography;  death;  Disabling pansclerotic morphea of childhood;  disease course;  Doppler flowmetry;  electrocardiogram;  enzyme linked immunosorbent assay;  female;  gangrene;  high risk patient;  hospitalization;  human;  immobilization;  joint contracture;  kinesiotherapy;  leg compression;  leg ulcer;  male;  medical literature;  morphea;  occupational therapy;  physical examination;  physiotherapy;  priority journal;  PUVA;  scleroderma;  sclerosis;  sepsis;  skin biopsy;  skin cancer;  skin ulcer;  small intestine;  squamous cell carcinoma;  tissue engineering;  transcutaneous nerve stimulation;  treatment outcome;  wound, Adolescent;  Adult;  Carcinoma, Squamous Cell;  Chronic Disease;  Collagen;  Combined Modality Therapy;  Contracture;  Female;  Humans;  Male;  Piperazines;  Purines;  Scleroderma, Localized;  Skin Neoplasms;  Skin Ulcer;  Skin, Artificial;  Sulfones;  Vasodilator Agents",
    abstract = "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 × 20 mg/day and repeated application of a porcine small intestinal submucosal acellular matrix. © 2007 Sage Publications."
}