We report a case of atrophoderma of Pasini and Pierini. We determined the glycosaminoglycan content in the involved skin. Dermatan sulfate content in the involved skin (1.88 μg uronic acid/mg dry skin) was greater than that in the uninvolved skin (1.05 μg uronic acid/mg dry skin). No significant differences in hyaluronic acid, chondroitin sulfate or heparan sulfate content between involved and uninvolved skin were observed. These results suggest that abnormal metabolism of dermatan sulfate may b...
Background : There are divergent opinions whether atrophoderma Pasini-Pierini (APP) is a nosologic entity or a primary atrophic morphea. Objective : Since usually s
Abstract Many different congenital and acquired skin diseases are seen along Blaschko's lines. In 1992, Moulin et al. [40] described five patients affected with a new clinical entity characterized by acquired atrophic band-like skin lesions showing hyperpigmentation. We report on a further patient with this skin disease. Characteristic features are hyperpigmented atrophoderma arranged in a pattern following the lines of Blaschko, with no preceding inflammation and no subsequent induration or scl...
Abstract Over a seventeen years period we observed 5 patients presenting with very similar lesions the equivalent of which we could not find in the literature. These were pigmented and more or less atrophic bands which exactly followed Blaschko's lines. The lesions appeared during childhood or adolescence, between the ages of 6 and 20 years and always were unilateral. In all cases they were located on the trunk where they formed a recumbent "S" pattern characteristic of Blaschko's lines. They of...
Linear atrophoderma of Moulin is a rare, acquired, linear dermatosis. We present a 17-year-old girl with multiple asymptomatic brownish atrophic plaques in a zosteriform distribution on the left side of the trunk. Clinical presentation and dermatopathology was compatible with the diagnosis of linear atrophoderma. Twenty years after its initial description by Moulin, there are yet a limited number of case reports and unanswered questions regarding this entity.
: We report an infant with depressed, hypopigmented, linear plaques of congenital onset on the lower extremity. The lesions were asymptomatic and the child was otherwise healthy. Despite the clinically obvious change in skin texture and color, histopathologic changes were subtle: a biopsy specimen showed hypopigmentation and a decrease in elastic fibers in the papillary and upper reticular dermis. Diagnoses considered included various congenital syndromes, idiopathic atrophoderma of Pasini and P...
Hyperpigmented atrophoderma arranged in a pattern following the lines of Blaschko and appearing during childhood or adolescence on the trunk or the limbs is a characteristic feature of linear atrophoderma of Moulin. We review 15 published reports and describe 4 additional cases. Histopathologically, there is no clear sign of atrophy found in specimens examined by light microscopy. It might well be argued that a focal reduction of subcutaneous fatty tissue contributes to the obvious clinical atro...
We present 3 unusual cases of deep linear, primary atrophic scleroderma, not preceded by inflammatory reaction and sclerosis, involving the subcutis and deeper tissues. These cases differ in the cours