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.     

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.     

Presence of Borrelia burgdorferi "Sensu Lato" in patients with morphea from the Amazonic region in Brazil

Background In the present study, Borrelia spirochetes were found in four (26,6%) out of 15 patients with Atrophoderma of Pasini and Pierini (IAPP) and lichen sclerosis et atrophicans (LSA) from the Brazilian Amazon Region. Material and methods Borreliosis was investigated by immunohistochemistry and focus floating microscopy for Borrelia burgdorferi in skin biopsy samples from 15 patients with both clinical and histopathology evidences compatible with Morphea, LSA, and IAPP. Results Spirochetes were detected by specific immunohistochemistry and focus floating microscopy for B. burgdorferi in samples from three patients. A limitation of our study was the fact that we were not able to isolate and culture these organisms. Conclusion Our data confirm the presence of borreliosis cases in the Amazon.     

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.     

Concomitant Unilateral Idiopathic Atrophoderma of Pasini and Pierini (IAPP) and Morphea

A 17-year-old black man suddenly developed an unilateral eruption of clinically apparent lesions of idiopathic atrophoderma of Pasini and Pierini (IAPP) and concomitant lesions of morphea. Histologically the lesions are indistinguishable. IAPP appears to be a variant of morphea.     

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.     

Morphoea in three siblings

Atrophoderma of Pasini and Pierini (APP) is an uncommon form of localized morphoea that occurs as superficial, hyperpigmented plaques distributed mainly on the trunk and proximal part of the limbs. There is little information about the influence of genetic and environmental factors on disease susceptibility and expression for localized scleroderma, although APP familial cases have been reported. We report three siblings without a family history of autoimmune disease presenting cutaneous lesions suggesting morphoea (APP variant).     

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.     

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.     

Phenylketonuria and scleroderma

Two mentally retarded siblings, one with severe segmental scleroderma and the other with atrophoderma of Pasini and Pierini, were found at the ages of 6 and 10 years to have phenylketonuria (PKU). The belief that a common pathomechanism exists between morphea and atrophoderma of Pasini and Pierini is supported by the case of the two siblings. Disorders in tryptophan metabolism can occur in both PKU and scleroderma. For a low phenylalanine diet to be effective in PKU, it has to be instituted at an early age. Phenylketonuria should be considered in infants and children with sclerodermatous skin lesions.     

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.     

Primary atrophoderma associated with hemiatrophy,essential teleangiectasie,lichen sclerosus and tumour of the skull

Summary A slow-growing, non invasive tumour of the skull situated at the inner side of the left temporal region, was found in a patient with mild ipsilateral facial hemiatrophy, marked contralateral hemiatrophy of the body and the extremities, and atrophoderma of Pasini and Pierini predominantly located on the same side of the body and the extremities. In addition, essential teleangiectasia and lichen sclerosus were found mainly within the atrophodermic plaques.On the basis of the circumstantial evidence it is suggested that the neurologic state caused by the skull tumour played an important role in the origin of hemiatrophy, primary atrophoderma and essential teleangiectasia.Lichen sclerosus is considered to be closely related to atrophoderma.

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Zusammenfassung Ein langsam größer werdender, nicht-invasiver Tumor mit Sitz in der linken Temporalgegend wurde bei einer Patientin, die im Gesicht an mäßiger Hemiatrophie, kontralateral ausgeprägter Körper- und Extremitäten-Hemiatrophie, sowie an einem Atrophoderma Pasini-Pierini hauptsächlich an der linken Körper- und Extremitätenseite litt, beobachtet.Als weitere Hautveränderungen fanden sich bei der Patientin essentielle Teleangiektasien und ein Lichen sclerosus, die hauptsächlich in den atrophodermischen Plaques lokalisiert waren.Es wurde auf die Rolle und Wichtigkeit der veränderten neurologischen Zustand, in unserer Patientin durch Schädel-Tumor verursacht, auf die Genese der Pasini-Pierinischen Atrophoderma sowie Teleangiektasien hingewiesen.Lichen sclerosus wurde als eine Krankheit, die in ihrer lokalen Reaktivität und Zustand nahezu Morphea und Atrophoderma liegt, angesehen.

     

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.”     

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

We report a 39-year-old Chinese man presenting with approximately 200 atrophic brownish patches for 10 years, whose clinical manifestation and pathological features were consistent with the findings of idiopathic atrophoderma of Pasini and Pierini. Another described case was a 62-year-old woman who had a gradually increasing asymptomatic hyperpigmented and depressed patch on her right lumbosacral region over a period of 7 years. At the time of consultation, the size of the lesion was approximately 27 x 23 cm.     

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.     

硬斑病患者180例临床特征及分类探讨

目的:分析硬斑病患者的临床特征及分类。方法:收集2010年1月至2021年7月就诊于复旦大学附属中山医院的180例硬斑病患者流行病学资料、临床表现和实验室数据进行回顾性分析。采用两独立样本 t检验比较性别间发病年龄差异,卡方检验比较不同性别、亚型间临床特征差异。 结果:180例患者中,女性123例...     

Unilateral congenital linear atrophoderma of the leg

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 Pierini, and especially, linear atrophoderma of Moulin. However, because of the significant clinical and histopathologic differences when compared to the aforementioned entities, our patient appears to have a unique presentation of congenital linear atrophoderma.     

Linear Atrophoderma of Moulin: A Distinct Entity?

Linear atrophoderma of Moulin (LAM) is a rare dermatologic disorder characterized by a hyperpigmented atrophoderma that consistently follows the lines of Blaschko. There are many clinical and histologic similarities between LAM, atrophoderma of Pasini and Pierini (APP), and morphea, and whether LAM represents part of a disease spectrum or its own distinct entity is debated. This case of a 16‐year‐old boy with LAM supports the hypothesis that LAM, APP, and morphea are a spectrum of disorders rather than unique entities. Although the patient's overall clinical picture supports a diagnosis of LAM with hyperpigmented, depressed lesions following the lines of Blaschko and perivascular lymphocytic infiltrate on biopsy, the bilateral presentation typical of APP, collagen entrapment of eccrine ducts typical of morphea, and changes in dermal collagen illustrate features spanning all three disorders, suggesting a relationship between these conditions that represents a spectrum of disease. Furthermore, a review of all reported cases of LAM in the literature suggests an evolving definition beyond what Moulin and colleagues originally described, including features related to those of APP and morphea.     

Glycosaminoglycans production by cultured skin fibroblasts from the Pasini and Cockayne-Touraine forms of dominant dystrophic epidermolysis bullosa

Qualitative and quantitative comparisons of glycosaminoglycans e (GAG) production were made on fibroblast lines cultured from the skin of six patients with the Pasini (albopapuloid) form of dominant dystrophic epidermolysis bullosa, six with the non-albopapuloid form (Cockayne-Touraine), eight lines from patients with simplex or recessive dystrophic epidermolysis bullosa and eight lines from normal individuals. A reasonable match of donor age and gender, site, and passage number was achieved. Contrary to an earlier report, the lines from the Pasini group were unexceptional in the amount of GAG they secreted and the proportions of sulfated and nonsulfated GAG showed no consistent difference from the Cockayne-Touraine or control lines. The Pasini lines secreted 77 +/- 18 (SEM) microgram GAG-uronic acid per 10(7) cells and the Cockayne-Touraine lines 81 +/- 12 micrograms at equivalent cells densities. Sulfated GAG represented averages of 19 +/- 4+ in Pasini lines, 17 +/- 5% in Cockayne-Touraine, and 14 +/- 3% in controls. These findings are consistent with current views of albopapuloid lesions as an unreliable clinical sign in epidermolysis bullosa and bring into question the validity of the Pasini entity.