Development of histologic features of scleroderma in congenital lesions.
BACKGROUND
Localized scleroderma has been reported after radiation therapy, but has never been reported to occur at the site of a congenital lesion.


METHODS
We present two patients, both with family histories of autoimmune disease, who reported unilateral hypopigmented areas on the trunk since birth. The areas remained asymptomatic and grew with the patients until adulthood when the areas became indurated then firm and showed hyperpigmentation.


RESULTS
Histologically, both lesions showed features of localized scleroderma with diffuse sclerosis of collagen and loss of periadnexal fat. There was a perivascular lymphoplasmocytic infiltrate with occasional eosinophils extending into the subcutaneous fat predominantly along fat septae, and diffuse loss of CD34+ stromal cell populations within the lesions.


CONCLUSIONS
We propose that somatic mutations affecting vessels may predispose to increased endothelial cell apoptosis. This could lead to the development of an autoimmune response in some individuals, and the areas of localized scleroderma may be markers of susceptibility to autoimmune disease.
Harford
Robert
R
Department of Dermatology and Pathology, National Naval Medical Center, Bethesda, MD, USA.
Smith
Kathleen J
KJ
Skelton
Henry
H
D000328:Adult; D000974:Antibodies, Antinuclear / Q000097:blood; D001327:Autoimmune Diseases / Q000473:pathology; D005260:Female; D006801:Humans; D007184:Incontinentia Pigmenti / Q000150:complications / Q000473:pathology*; D008297:Male; D011247:Pregnancy; D011248:Pregnancy Complications; D012594:Scleroderma, Localized / Q000097:blood / Q000150:complications / Q000473:pathology*
D000974:Antibodies, Antinuclear