Departments of Dermatology, Venereology, Allergology and Immunology, Dessau Medical Center, Brandenburg Medical School Theodor Fontane and Faculty of Health Sciences Brandenburg, Dessau, Germany.
Department of Dermatology, Jagiellonian University Medical College, Krakow, Poland.
J Eur Acad Dermatol Venereol. 2022 Nov;36(11):2190-2194. doi: 10.1111/jdv.18260. Epub 2022 Jun 7.
Atrophic papulosis is a very rare vascular disease of unknown pathogenesis, mostly described by case reports.
To assess demographic data and prognosis in patients with atrophic papulosis.
A single-centre study was performed on a series of 105 patients with atrophic papulosis, diagnosed 2000-2021. Patients were referred and diagnosed at the evaluation centre and patients' clinical data were provided by the Degos Support Network and evaluated by the authors for confirming the diagnosis of skin lesions and fulfilling the diagnostic criteria for a malignant subset. A unique set of variables were collected from all patients.
The mean age of disease onset was 33.3 ± 18.3 years and the male-to-female ratio was 1:1.6. The family history rate was 8.1%. The classification into a benign, merely cutaneous disease (benign atrophic papulosis), and malignant atrophic papulosis, associating cutaneous and visceral lesions was confirmed due to their striking prognostic difference. Benign atrophic papulosis was detected in 41% of the patients with no deaths occurring throughout the follow-up period (median 3.00 years; range 0.13-23). Malignant atrophic papulosis was reported in 59% of patients with 47.5% multisystemic involvement and a median skin lesion onset to systemic symptoms duration of 0.54 years (-6 to 20). The gastrointestinal tract and central nervous system were equally involved; however, the neurological involvement-caused death rate was slightly higher. The disease-specific mortality rate of malignant atrophic papulosis was 22.6%.
Atrophic papulosis presents with a striking prognostic difference of benign - merely cutaneous - involvement or quickly developing - into less than 1 year - malignant subset, associating cutaneous and visceral lesions and multiorgan involvement in 1/2 of the patients, which leads to premature, disease-specific mortality in 1/4 of the cases. Central nervous system and gastrointestinal tract complications are the major reasons for disease-specific death. Over the years, the diagnosis of severe nervous system involvement has become more common.
萎缩性丘疹病是一种非常罕见的血管疾病，其发病机制尚不清楚，大多通过病例报告进行描述。
评估萎缩性丘疹病患者的人口统计学数据和预后。
对 2000 年至 2021 年期间在一家医疗中心确诊的 105 例萎缩性丘疹病患者进行了单中心研究。患者在评估中心就诊和诊断，Degos 支持网络提供了患者的临床数据，作者对这些数据进行了评估，以确认皮肤病变的诊断并符合恶性亚型的诊断标准。从所有患者中收集了一组独特的变量。
疾病发病的平均年龄为 33.3±18.3 岁，男女比例为 1:1.6。有家族史的患者占 8.1%。由于预后差异显著，将其分为良性、单纯皮肤疾病（良性萎缩性丘疹病）和恶性萎缩性丘疹病，合并皮肤和内脏病变。良性萎缩性丘疹病患者占 41%，在整个随访期间无死亡（中位随访时间为 3.00 年；范围为 0.13-23 年）。恶性萎缩性丘疹病患者占 59%，有 47.5%的患者有系统性受累，皮肤病变发病至出现系统性症状的中位时间为 0.54 年（-6 至 20 年）。胃肠道和中枢神经系统同样受累；然而，神经受累导致的死亡率略高。恶性萎缩性丘疹病的疾病特异性死亡率为 22.6%。
萎缩性丘疹病具有明显的预后差异，良性-单纯皮肤-受累或在不到 1 年内迅速发展为恶性亚型，合并皮肤和内脏病变以及 1/2 的患者多器官受累，导致 1/4 的患者出现疾病特异性死亡。中枢神经系统和胃肠道并发症是导致疾病特异性死亡的主要原因。近年来，严重神经系统受累的诊断变得更加常见。
