Sharma Shriya, Adhikari Aakriti, Yadav Sumit Kumar, Mainali Gaurab, Rajkarnikar Ruja
Nepalese Army Institute of Health Sciences, Kathmandu, Nepal.
Department of Internal Medicine, Shree Birendra Hospital, Kathmandu, Nepal.
Ann Med Surg (Lond). 2022 Jul 12;80:104164. doi: 10.1016/j.amsu.2022.104164. eCollection 2022 Aug.
Sarcoidosis is a common, multisystemic non-caseating granulomatous disease of unknown etiology with cutaneous lesions present in about one-fourth of patients. Darier Roussy sarcoidosis is a rare variant of sarcoidosis with distinct cutaneous presentation characterized by multiple deep-seated nodules on the trunk and extremities which could either be asymptomatic or may present mild tenderness.
A case of 35 yrs male with cough and fever for 3 months was initially diagnosed as a case of tubercular lymphadenitis and started with ATT following which ATT-associated cutaneous adverse drug reaction was suspected due to development of rashes with generalized redness and mild itching a few weeks after starting ATT. He then developed multiple, skin-colored, deep-seated, subcutaneous lesions over the legs then over the arms, forearms, thigh, and trunk. FNAC and histopathological examination of the lesions revealed non-caseating granulomas composed localized to the subcutaneous tissue. A diagnosis of subcutaneous sarcoidosis was made. Subsequently, steroid therapy was started.
Clinical manifestations of sarcoidosis range from asymptomatic (mostly) to progressive and relapsing disease. A family history of the disease raises the risk; those with one afflicted first-degree relative face a 3.7-fold increase in risk. Sarcoidosis is diagnosed based on three key criteria: a consistent clinical presentation, the discovery of non-necrotizing granulomatous inflammation in one or more tissue samples with confirmed histology, and the elimination of other origins of granulomatous disorders. . Diagnosis should be confirmed with a biopsy of the lesion, with the histological finding of non-caseating granuloma.
Clinically localized subcutaneous sarcoidosis can be confused with ATT induced drug reaction due to the difficulties in diagnosing granulomatous skin disease. The prognosis is good with subcutaneous disease and if there are no disfiguring skin lesions or other critical organ involvement, corticosteroid therapy might suffice.
结节病是一种常见的多系统非干酪样肉芽肿性疾病，病因不明，约四分之一的患者有皮肤损害。 Darier Roussy结节病是结节病的一种罕见变异型，具有独特的皮肤表现，其特征为躯干和四肢出现多个深部结节，这些结节可能无症状，也可能有轻度压痛。
一名35岁男性，咳嗽、发热3个月，最初被诊断为结核性淋巴结炎并开始抗结核治疗，开始抗结核治疗几周后，因出现皮疹、全身发红和轻度瘙痒，怀疑是抗结核药物相关的皮肤不良反应。随后，他的腿部、手臂、前臂、大腿和躯干出现多个肤色、深部、皮下病变。对病变进行细针穿刺抽吸活检（FNAC）和组织病理学检查，发现皮下组织有局限性非干酪样肉芽肿。诊断为皮下结节病。随后开始使用类固醇治疗。
结节病的临床表现从无症状（大多数情况）到进行性和复发性疾病不等。该病家族史会增加患病风险；有一个患病一级亲属的人患病风险增加3.7倍。结节病的诊断基于三个关键标准：一致的临床表现、在一个或多个经组织学证实的组织样本中发现非坏死性肉芽肿性炎症，以及排除肉芽肿性疾病的其他病因。诊断应通过病变活检来确认，组织学检查发现非干酪样肉芽肿。
临床上局限性皮下结节病可能因肉芽肿性皮肤病诊断困难而与抗结核治疗引起的药物反应相混淆。皮下疾病预后良好，如果没有毁容性皮肤病变或其他重要器官受累，皮质类固醇治疗可能就足够了。
