Clinical features and classification of 180 patients with morphea / 中华皮肤科杂志

Objective:To determine classification and clinical features of morphea.Methods:A retrospective analysis was conducted on epidemiological information about clinical manifestations of and laboratory data from 180 patients with morphea, who visited Zhongshan Hospital, Fudan University from January 2010 to July 2021. Two-independent-sample t test was used to compare the age at onset between genders, and chi-square test to analyze differences in clinical characteristics between different genders and subtypes. Results:Among the 180 patients, 123 were females and 57 were males, with a male-to-female ratio of 1∶2.16. The age at onset of morphea was 28.69 ± 17.97 years for female patients, and 29.90 ± 20.67 years for male patients. Among them, linear morphea was the most common type in this study (68 cases, 37.78%), followed by plaque morphea (63 cases, 35.00%), mixed morphea (28 cases, 15.56%) and deep morphea (21 cases, 11.67%). The disease occurred in all age groups, but the age at onset significantly varied among different clinical subtypes ( F = 5.95, P < 0.001). No significant difference was observed in the age at onset or proportion of clinical subtypes between genders ( F = 0.15, P = 0.696; χ2 =2.88, P = 0.410). Atrophoderma of Pasini and Pierini (APP) was very common (62 cases, 34.44%) in the 180 patients, which mainly manifested as plaques or linear lesions, and 26 out of 45 patients with plaque APP and 11 out of 17 with linear APP were both accompanied by other subtypes of morphea. Among the 75 patients tested for autoantibody profiles, 34 (45.33%) presented with positive results. More diverse types of autoantibodies were found in female patients compared with male patients, and antinuclear antibodies, anti-SSA and anti-SSB antibodies were the most common types. There were various types of comorbidities in female patients, but lichen sclerosus et atrophicus and vitiligo were the most common comorbidities in both genders. Conclusion:High incidence and frequent co-occurrence with other subtypes of APP may be the characteristics of Chinese patients with morphea, and it is recommended to classify morphea into plaque, linear, deep and mixed subtypes.

Atrophoderma of pasini and pierini on the head and trunk of a 32 year-old male: A case report

@#Atrophoderma of Pasini and Pierini (APP) is an idiopathic disorder of dermal atrophy, presenting as round or oval depressed lesions most commonly observed on the trunk and extremities. Herein, we report the first case of APP initially appearing on the head. Histopathologic examination of lesional skin demonstrated a decrease in elastic fibers. Serologic tests for Borrelia and antinuclear antibody titers were negative. There was no noted improvement after 6 weeks tretament with Hydroxycholoroquine 200 mg/tab BID. Definitive disease etiology and treatment of APP remains to be elucidated.

Zosteriform Atrophoderma of Pasini and Pierini / 대한피부과학회지

Atrophoderma of Pasini and Pierini is characterized by sharply demarcated, slightly depressed blue-brown plaques, arising within the areas of the normal skin. The lesions are usually symmetrically distributed on the trunk, particularly on the back, and to a lesser extent on the extremities. A 14-year-old boy developed well-demarcated depressed plaques in a zosteriform distribution, along the left side of his shoulder, back, and arm, without any symptoms. A biopsy taken from the border between the depressed plaque and the normal-looking skin on the back showed evident dermal atrophy with decreased dermal thickness and thickening of collagen bundles in the depressed plaque, compared to the normal-looking skin, which were consistent with atrophoderma of Pasini and Pierini. Herein, we report an unusual case of zosteriform atrophoderma of Pasini and Pierini.

Zosteriform Atrophoderma of pasini and Pierini / 대한피부과학회지

A 13-year-old girl developed rice to nut-sized, bluish to slate-gray colored, depressed patches in a zosteriform distribution along the right side of her upper back and arm without any symptoms. Microscopically, the lesions showed a normal-appearing epidermis, dermis, and subcutaneous fat tissue except for a thinning of collagen fibers and widening of intervals between them in the lower dermis. There have been no changes to the skin lesions during the last 2 years.

Atrophoderma of pasini and pierini / 대한피부과학회지

We report a case of atrophoderma of Pasini and Pierini in which the characteristic soft atrophic patch with cliffdrop border developed on the neck and two well-demarcated indurated atrophic patches were on both shoulders. We took biopsies from centers and margins of each lesion. Histipathologic findings from atrophic center showed marked dermal atrophy comparing to that from margin of the lesion on the neck, but the atrophic center and margin of the lesion on the shoulder showed morphea-like features without, distinct differences between two.

A case of idiopathic progressive atrophoderma of pasini and pierini / 대한피부과학회지

Idiopathic progressive atrophoderma of Pasini and Pierini(IPAPP) is charcterized by sharply dermatcated, atrophic and slate-gray to brownish patches. We report a 17-year-old female with well-defined, atrophic, and brown colored patched on the abdomen, flank, and back. Histologic finding showed marked thining of dermis with thichening of collagen fibers and fragmentation of elastic fibers.

Idiopathic Progressive Atrophoderma of Pasini and Pierini / 대한피부과학회지

We report a case of idiopathic progressive atrophoderma of Pasini and Pierini in which the characteristics atrophic paches developed on the lumbar area and new lesions have a zosteriform distribution in lumbar area, scapular area and heels. This 32-year-old male patient who has had above lesions for 15 yeara, was first seen in September 1985. Histologic findings of this patient are atrophy in epidermis, tightly packed collagen bundles and moderatly decreased elastic fibers in the dermis. We observed this patient without treatment and 5 month later, lesions did not progress.