Disaccharide analysis of skin glycosaminoglycans in atrophoderma of Pasini and Pierini

Abstract

There are divergent opinions as to whether atrophoderma of Pasini and Pierini (APP) is a nosologic entity or a primary atrophic morphoea. In this study, we used high performance liquid chromatography to analyse the skin disaccharide contents of glycosaminoglycan (GAG) in two patients with APP and compared the results with those from a typical atrophic morphoea patient. Perilesional uninvolved skin was used as a control in each patient. In the atrophic phase morphoea, both the total amount of disaccharide per skin punch-biopsy and the amount of DeltaDi-4S(DS) - the main disaccharide unit of dermatan sulphate - per mg dry weight were increased. These changes were consistent with sclerotic phase morphoea. In contrast, the total amount of disaccharide per skin punch-biopsy was decreased and the amount of DeltaDi-4S(DS) per mg dry weight was decreased or unchanged in APP. Our results suggest that GAG metabolism in APP may be unique and quite different from that in morphoea.

Congenital Atrophoderma of Pasini and Pierini

“…Moreover, histological findings such as dermal atrophy and usual absence of dermal sclerosis of IAPP are different from those of morphea (1). A report that analyzed glycosaminoglycans in IAPP and morphea showed decreased amount of glycosaminoglycans in IAPP but increased amount in morphea, which suggests that glycosaminoglycans metabolism in IAPP may be unique and quite different from that in morphea (12). From this point of view, our patient could be diagnosed as IAPP.…”

Hydroxychloroquine as a treatment for atrophoderma of Pasini and Pierini

“…It has also been postulated that APP is a variant of morphea 3,5 . However, some immunoflourescence data and analysis of skin glycosaminoglycans contradicts this hypothesis 7,8 . While there is no universally accepted therapy for morphea, hydroxychloroquine has been employed with some success 9 …”

Disaccharide analysis of skin glycosaminoglycan in lichen myxedematosus

“…1,2 The procedures for RNA extraction and polymerase chain reactions (PCR) are described before. 1,2 The procedures for RNA extraction and polymerase chain reactions (PCR) are described before.…”

“…As common for nuts, the risk of severe allergic reactions is high. 1,4,10 There have, however, also been reports of anaphylaxis among individuals with an isolated allergy to macadamia nut. 10 macadamia nuts.…”