Congenital idiopathic atrophoderma of Pasini and Pierini

Idiopathic atrophoderma of Pasini and Pierini is a disorder of dermal atrophy. There is a female predominance and almost never does the condition present at birth. Histopathological examination reveals attenuated dermis. We report a case of a healthy male born with a plaque of idiopathic atrophoderma of Pasini and Pierini.     

Unilateral atrophoderma of Pasini and Pierini

Atrophoderma of Pasini and Pierini usually appears as isolated, round to oval depressed areas of otherwise normal-appearing skin. The clinical appearance has been likened to "footprints in the snow" or depressions with "cliff drop" borders. The condition is of unknown cause and most commonly occurs on the back, but it is occasionally seen elsewhere on the body. A literature search revealed only one previous report of unilateral atrophoderma of Pasini and Pierini.     

Zosteriform atrophoderma of Pasini and Pierini

Astophoderma of Pasini and Pierini is generally regarded as an atrophic variant of morphoea. It arises most commonly on the trunk and abdomen. We describe a 53-year-old man who had a 12-year history of unilateral atrophoderma of Pasini and Pierini affecting the left side of his trunk in a zosteriform distribution. Within these clinically atrophic lesions, there were localized areas of induration which were typical of morphoea. The patient also had a preceding history of balanitis xerotica obliterans. The occurrence of atrophoderma of Pasini and Pierini, morphoea and lichen sclerosus within the same individual supports the view that these conditions arc closely related, and suggests that common factors may be involved in their aetiologies.     

Idiopathic atrophoderma of Pasini and Pierini. Study of 4 cases

Idiopathic atrophoderma of Pasini and Pierini (IAPP) is an infrequent form of cutaneous atrophy, of unknown etiology. We describe the clinical and histological symptoms of four women with IAPP. Three of them had lesions on the back, while the fourth had them on the buttocks. The histological study of two patients revealed a variable degree of collagen alteration (sclerosis and hyalinization). In the other patients, the dermis was apparently normal. There is discussion about whether IAPP is a distinct entity or is related to morphea. Both sets of symptoms, although separate, could form part of the same spectrum within the fibrosing type of dermatoses. We found no positive serology for B. burgdorferi in our patients.     

Combined high-frequency and Doppler ultrasound in early diagnosis of atrophoderma of Pasini and Pierini

Atrophoderma of Pasini and Pierini is a skin disorder affecting dermal collagen and is clinically characterized by well-defined plaques of depressed skin. Histopathological changes are subtle, and in most cases, the diagnosis requires a comparative study with healthy skin from the same anatomical site. High frequency ultrasound is a useful imaging method for diagnosis of atrophic skin changes. A case is presented in which ultrasound can support the clinical and the histopathological diagnosis of atrophoderma of Pasini and Pierini.     

Disaccharide analysis of the skin glycosaminoglycans in systemic sclerosis

The disaccharides constituting chondroitinase-digestible glycosaminoglycan (GAG) in the skin lesions of patients with systemic sclerosis were determined using high-performance liquid chromatography (HPLC). In scleroderma there was an increase in the amount of delta Di-4S(DS), the main disaccharide unit of dermatan sulphate, and a decrease in delta Di-HA, the disaccharide unit of hyaluronic acid, as compared with normal skin from a similar site. The distribution pattern of the main disaccharides constituting chondroitin sulphate and dermatan sulphate in scleroderma differed from that in scars or scleredema.     

Disaccharide analysis of the skin glycosaminoglycans in patients with Werner's syndrome

The disaccharide content of the chondroitinase-digestible glycosaminoglycans (GAGs) extracted from 6–mm skin punch biopsies from the atrophic and sclerotic skin of two patients with Werner's syndrome (WS) were determined using high-performance liquid chromatography after 1–phenyl-3–methyl-5–pyrazolone labelling. The total amount of main disaccharides was significantly decreased in the atrophic lesions of WS. In the atrophic forearm skin, the decrease in the main disaccharide unit of hyaluronic acid, ΔDi-HA, and the increase in the ratio of the main disaccharide unit of dermatan sulphate, ΔDi-4S, to ΔDi-HA were significant vs. normal control (P <0.01 and 0.05, respectively). The sclerotic skin showed an increase in ΔDi-4S (DS) (P < 0.05) and a decrease in ΔDi-HA (P < 0.02) compared with normal controls, as well as a significantly higher ratio of ADi-4S (DS)/ΔDi-HA compared with normal controls (P < 0.0002) and systemic sclerosis patients (SSc; P < 0.02). No other statistical difference was found in the amount of each main disaccharide unit between the sclerotic skin of WS and SSc. Histological examination revealed that the atrophic skin showed thinning of the dermis with a slight increase of fine collagen bundles, whereas the sclerotic skin demonstrated a thickened dermis with prominent deposition of fine collagen bundles in the deep dermis. In SSc, thickening of the whole dermis, composed of hyalinized or swollen collagen bundles, was found. These results suggest mat alterations of disaccharide components in WS may differentiate the atrophic skin changes from the sclerotic skin changes, while the mechanisms for abnormal fibrosis remain to be elucidated.     

Generalized Lenticular Atrophoderma of Pasini and Pierini

We present a 16‐year‐old boy with multiple, well‐circumscribed, atrophic, light‐brown patches on his neck, chest, and back. The authors believe that it represents an unusual presentation of atrophoderma of Pasini and Pierini and suggest the designation “generalized lenticular APP.”     

Atrophoderma of Pasini and Pierini: a clinical and histopathological study

Background: Idiopathic atrophoderma of Pasini and Pierini (IAPP) usually manifests as one or multiple depressed and hyperpigmented patches, with a predilection to the trunk. No diagnostic changes are usually seen on histology. Elastic stains often reveal no abnormalities. Objective: To review our cases of IAPP, describe their clinical and histological findings and compare them with the literature. Methods: Retrospective review of IAPP cases who presented to our institution between 1994 and 2006. Results: From a total of 16 patients, only 19% displayed hyperpigmented lesions, while 81% had either hypopigmented (9/16) or skin‐colored (4/16) lesions. The sites predominantly affected were the lower extremities (62.5%), followed by the upper extremities and trunk. Only in one patient was IAPP co‐existent with morphea. Histology revealed no diagnostic abnormalities; however, elastic stains showed a spectrum of changes ranging from normal to severe diminution and fragmentation of elastic fiber network. Conclusions: Our study shows several new aspects of IAPP. Clinically, the lesions were most commonly hypopigmented and involved predominantly the extremities. Histologically, IAPP exhibited a spectrum of alterations in elastic fibers. The most prevalent form of IAPP in our country seems to be unassociated with morphea.