Abbas Hafsa, Aslam Ahsan, Saad Muhammad, Niazi Masooma, Chilimuri Sridhar
Department of Medicine, Division of Gastroenterology, Bronxcare Hospital Center, Bronx, NY 10457, USA.
Department of Medicine, IU Health University Hospital, Indianapolis, IN 46202, USA.
Case Rep Dermatol Med. 2019 Jul 3;2019:6830862. doi: 10.1155/2019/6830862. eCollection 2019.
Cytophagic histolytic panniculitis (CHP) is a clinical disorder characterized by nodular panniculitis of the subcutaneous adipose tissue. It was first described in 1980 by Winkelmann. Histologically it is described as an infiltration of the adipose tissue by T- lymphocytes and phagocytic macrophages (also known as "bean bag cells"). Most of the cases are reported under the age of 50 and is a rare cause of panniculitis. We report a case of CHP in a young patient who presented to our emergency room (ER).
A 39-year-old African American woman who presented to our hospital with lethargy, progressive confusion, and generalized rash involving both lower extremities of 1 week duration. She had a history of pancytopenia and focal proliferative and membranous lupus nephritis classes 3 and 5. Her physical examination was remarkable for bilateral lower extremity pitting edema and a desquamating rash on both of her legs. The Nicolsky sign was positive. She was noted to be hypotensive and was started on intravenous fluids and broad spectrum antibiotics. Routine laboratory tests revealed severe pancytopenia, with a hemoglobin of 3.9 g/dl, white blood cell count 600/ul, and platelet count of 11000/ul. Within an hour of arrival to the ER she developed acute respiratory failure. She was intubated and placed on mechanical ventilation. She developed shock requiring vasopressors. No imaging could be done due to her unstable condition. Four hours after her initial presentation she developed asystole and expired. Postmortem histopathology of the adipose tissue revealed CHP.
CHP can be rapidly fatal. The treatment involves high dose of intravenous steroids and immunosuppressants such as cyclosporine.
噬血细胞性组织细胞性脂膜炎（CHP）是一种以皮下脂肪组织结节性脂膜炎为特征的临床病症。它于1980年由温克尔曼首次描述。组织学上，它被描述为脂肪组织被T淋巴细胞和吞噬性巨噬细胞（也称为“豆袋细胞”）浸润。大多数病例报告发生在50岁以下，是脂膜炎的罕见病因。我们报告一例年轻患者的CHP病例，该患者前来我们的急诊室就诊。
一名39岁非裔美国女性，因嗜睡、进行性意识模糊以及累及双下肢持续1周的全身性皮疹前来我院就诊。她有全血细胞减少病史，以及3级和5级局灶增生性和膜性狼疮性肾炎。她的体格检查显示双侧下肢凹陷性水肿以及双腿脱皮性皮疹。尼氏征阳性。她被发现血压低，开始接受静脉输液和广谱抗生素治疗。常规实验室检查显示严重全血细胞减少，血红蛋白为3.9g/dl，白细胞计数600/ul，血小板计数11000/ul。到达急诊室后一小时内，她出现急性呼吸衰竭。她被插管并接受机械通气。她出现休克，需要使用血管加压药。由于她病情不稳定，无法进行影像学检查。首次就诊4小时后，她出现心搏停止并死亡。脂肪组织的尸检组织病理学显示为CHP。
CHP可能迅速致命。治疗包括大剂量静脉注射类固醇和免疫抑制剂，如环孢素。
