Bottoni U, Innocenzi D, Bonaccorsi P, Carlesimo M, Faina P, Richetta A, Cugini P, Calvieri S
Department of Dermatology, University of Rome La Sapienza, Italy.
J Eur Acad Dermatol Venereol. 2002 Sep;16(5):500-3. doi: 10.1046/j.1468-3083.2002.00546.x.
Darier's erythema annulare centrifugum (EAC) is often associated with infectious, autoimmune or neoplastic disease, nevertheless, most cases of EAC remain unexplained. We report a case of EAC with neonatal onset and a clinical course of over 20 years. The patient presented from the 6th day of life with erythematous papules that enlarged centrifugally to form figurate, annular or policyclic plaques involving the entire cutaneous surface. The clinical picture had a chronic course, disappearing only during unrelated febrile episodes. Histologic examination revealed a normal epidermis and a dense perivascular lymphohistiocytic infiltrate and numerous eosinophils in the superficial and reticular dermis. Today the patient is 24 years old and his lesions are still unchanged. Recently, clinical evaluation revealed a diffuse sderodermic induration. A second biopsy showed a diffuse thickening of collagen fibers in the dermis. To our knowledge, this is the second report of EAC with neonatal onset and such a long and peculiar clinical course.
Darier 离心性环状红斑(EAC)常与感染性、自身免疫性或肿瘤性疾病相关,然而,大多数 EAC 病例病因不明。我们报告一例新生儿期起病且病程超过 20 年的 EAC 病例。该患者自出生第 6 天起出现红斑丘疹,离心性扩大形成图形、环状或多环状斑块,累及整个皮肤表面。临床表现呈慢性病程,仅在无关的发热发作期间消失。组织学检查显示表皮正常,真皮浅层和网状层有密集的血管周围淋巴细胞组织细胞浸润及大量嗜酸性粒细胞。如今患者 24 岁,其皮损仍无变化。最近,临床评估发现有弥漫性硬皮病样硬结。第二次活检显示真皮中胶原纤维弥漫性增厚。据我们所知,这是第二例新生儿期起病且有如此漫长而特殊临床病程的 EAC 报告。