Disabling pansclerotic morphea of childhood poses a high risk of chronic ulceration of the skin and squamous cell carcinoma.
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.
Wollina
Uwe
U
Department of Dermatology and Allergology, Hospital Dresden Friedrichstadt, Academic Teaching Hospital of the Technical University of Dresden, Dresden, Germany. wollina-uw@khdf.de
Buslau
Michael
M
Heinig
Birgit
B
Petrov
Ivailo
I
Unger
Eleonore
E
Kyriopoulou
Eugenia
E
Koch
André
A
Köstler
Erich
E
Schönlebe
Jaqueline
J
Haroske
Gunther
G
Doede
Thorsten
T
Pramatarov
Kyrill
K
D000293:Adolescent; D000328:Adult; D002294:Carcinoma, Squamous Cell / Q000209:etiology*; D002908:Chronic Disease; D003094:Collagen / Q000627:therapeutic use; D003131:Combined Modality Therapy; D003286:Contracture / Q000209:etiology; D005260:Female; D006801:Humans; D008297:Male; D010879:Piperazines / Q000627:therapeutic use; D011687:Purines / Q000627:therapeutic use; D012594:Scleroderma, Localized / Q000150:complications* / Q000628:therapy; D000068677:Sildenafil Citrate; D012878:Skin Neoplasms / Q000209:etiology*; D012883:Skin Ulcer / Q000209:etiology*; D017768:Skin, Artificial; D013450:Sulfones / Q000627:therapeutic use; D014665:Vasodilator Agents / Q000627:therapeutic use
C117898:Apligraf; D010879:Piperazines; D011687:Purines; D013450:Sulfones; D014665:Vasodilator Agents; D003094:Collagen; D000068677:Sildenafil Citrate