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.”     

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.     

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.     

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.     

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

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 be involved in the pathogenesis of atrophoderma; this pattern has been observed in systemic or localized scleroderma.     

A Case of Atrophoderma of Pasini and Pierini Associated with Borrelia burgdorferi Infection Successfully Treated with Oral Doxycycline

Atrophoderma of Pasini and Pierini is a form of dermal atrophy that manifests as either single or multiple, sharply demarcated, hyperpigmented, non-indurated patches. These patches are marked by a slight depression of the skin, with an abrupt edge (i.e., the "cliff-drop" borders), usually located on the backs of adolescents or young adults. The pathophysiology of the disease is unknown, but some authors have suggested a role of Borrelia burgdorferi infection. A 35-year-old woman visited our department because of asymptomatic, hypopigmented, depressed patches on her chest and back lasting for three months. Laboratory evaluations were normal, except for positive serum antibodies to Borrelia burgdorferi. Histologic examination revealed a significantly decreased thickness of the dermis. The patient underwent treatment with oral doxycycline 200 mg/day for six weeks, after which the depth of depression was improved. Herein, we report a case of atrophoderma of Pasini and Pierini, associated with Borrelia burgdorferi infection, successfully treated with oral doxycycline.     

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.     

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.     

Disaccharide analysis of skin glycosaminoglycans in atrophoderma of Pasini and Pierini

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.     

Atrophoderma (Pasini-Pierini)

A patient with atrophoderma (Pasini-Pierini) was studied. Microscopic examination showed small collections of mononuclear cells around dermal blood vessels. Electron microscopic study demonstrated macrophages and lymphocytes around vessels and between fibers in the dermis; the epidermis, dermis, collagen, and elastic fibers appeared normal. Monoclonal antibody studies of the cells in the perivascular infiltrate demonstrated cells reacting with anti-Leu-1 (pan-T-cell antibody), anti-Leu-3a (the helper/inducer T-cell antibody), and OKM 1 antibody-reacting cells (macrophages). Direct immunofluorescent studies showed IgM and C3 staining in the small blood vessels of the papillary dermis, scattered IgM cytoids at the basement membrane, and focal fibrinogen in the mid-dermis. Mononuclear cells in the perivascular infiltrate, similar in type and percentage concentration, have been demonstrated also in patients with anetoderma, another rare atrophic cutaneous disorder. Macrophages and T lymphocytes around papillary dermal blood vessels may play a role in the pathogenesis of atrophoderma and anetoderma.     

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.