Santosh Tummidi, Patra Suman, Goel Garima, Rupla Richa
Department of Pathology, AIIMS Mangalagiri, AP, India.
Department of Dermatology, AIIMS, Jodhpur, Rajasthan, India.
Indian Dermatol Online J. 2021 Sep 10;12(5):741-744. doi: 10.4103/idoj.IDOJ_383_20. eCollection 2021 Sep-Oct.
Adult-onset Still's disease (AOSD) is a rare systemic inflammatory condition classically consists of high spikes of fever, morbilliform evanescent skin rash, arthritis, neutrophilic leukocytosis, and multiple organ involvement. However, atypical form of skin rashes has been described with few specific clinical and histopathological patterns that can help in making the diagnosis. A 25-year-old female presented with high spikes of fever, severe debilitating arthritis of peripheral joints, and dusky erythematous flagellate rashes over trunk and proximal limbs. Skin punch biopsy from the cutaneous lesion revealed dyskeratotic keratinocytes in upper epidermis and stratum corneum along with acute inflammatory infiltrate in the dermis: a finding that recently has been found to be constantly associated with this specific pattern of rash of AOSD. The presence of necrotic keratinocytes in upper epidermis and a dermal infiltrate of neutrophils along with characteristic clinical scenario may facilitate earlier diagnosis of AOSD.
成人斯蒂尔病(AOSD)是一种罕见的全身性炎症性疾病,典型表现为高热、一过性麻疹样皮疹、关节炎、中性粒细胞增多以及多器官受累。然而,已经描述了非典型皮疹形式,其具有一些有助于诊断的特定临床和组织病理学特征。一名25岁女性出现高热、外周关节严重致残性关节炎,以及躯干和近端肢体出现暗红色鞭状皮疹。对皮肤病变进行的皮肤打孔活检显示,上表皮和角质层有角化不良的角质形成细胞,真皮有急性炎症浸润:这一发现最近被发现与AOSD这种特定皮疹模式始终相关。上表皮坏死角质形成细胞的存在以及真皮中性粒细胞浸润,再加上特征性临床情况,可能有助于AOSD的早期诊断。