A Case of Atrophoderma of Pasini and Pierini: Analysis of Glycosaminoglycan of the Lesional Skin

Abstract

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 micrograms uronic acid/mg dry skin) was greater than that in the uninvolved skin (1.05 micrograms 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 be involved in the pathogenesis of atrophoderma; this pattern has been observed in systemic or localized scleroderma.

Unilateral Congenital Linear Atrophoderma of the Leg

“…In contrast to the normal epidermis and dermis and lack of inflammation originally reported, these authors describe such variable features as: thickened collagen and sclerosis (8,10,11,14,16), decreased elastic fibers (16), inflammation (13,15,18), and hypopigmentation (16). We believe that those instances exhibiting vacuolar alteration and a mononuclear infiltrate of inflammatory cells (8,9,14), actually represent morphea or APP following the lines of Blaschko, as has previously been reported (1,3,4,19,20), and not LAM. Some authors (17,21) have even suggested the reclassification of some of the patients originally reported as Blaschkolinear APP by Murphy et al (1) and Tajima et al (3).…”

“…The major abnormal findings in our patient are as follows: (1) congenital onset; (2) stable, slightly depressed linear plaques; (3) a lack of symptomatology or attendant malformations; and (4) subtle histopathologic changes of the affected epidermis and papillary dermis accounting for the clinical atrophy.…”

“…Dermatoses that may present in a linear distribution include lichen striatus, so‐called Blaschko dermatitis, morphea, lichen sclerosus et atrophicus (LS & A), atrophoderma Pasini and Pierini (APP), and linear atrophoderma of Moulin (LAM) (1–7). We present a child with congenital, linear, hypopigmented atrophoderma of the lower extremity, which shares some clinical features with LAM.…”

Disaccharide analysis of skin glycosaminoglycans in atrophoderma of Pasini and Pierini

“… 1–3 Tajima et al . 20 reported the increase of DS and total GAG content per mg dry weight in the involved skin of APP. With regard to this discrepancy, it is possible that Tajima's case was a type of APP of closely related to LSc and that our cases were APP of true unique atrophy.…”

Two uncommon cases of idiopathic atrophoderma of pasini and pierini: Multiple and giant

“…Previous studies suggested that genetic factors, neurogenic cause, immunological factors, and abnormal metabolism of dermatan sulfate may play a role in the pathogenesis of IAPP. [2][3][4][5] The role of Borrelia burgdorferi remains controversial. [6] IAPP is classically thought of as an idiopathic atrophy of the dermis.…”