Jacquin-Porretaz C, Devalland C, Delapparent T, Nardin C, Dupond A-S
Service de dermatologie, hôpital Nord Franche-Comté, 100, route de Moval 90400 Trévenans, France.
Service d'anatomopathologie, hôpital Nord Franche-Comté, 100, route de Moval, 90400 Trévenans, France.
Ann Dermatol Venereol. 2019 Sep;146(8-9):571-576. doi: 10.1016/j.annder.2019.04.023.
Idiopathic granulomatous mastitis (IGM) is a rare, chronic, granulomatous, inflammatory disorder with potentially misleading clinical and radiological features. IGM is diagnosed after exclusion of infectious or tumoral diseases. Herein we report a case of erythema nodosum associated with IGM, which serves as a reminder that erythema nodosum may constitute an extra-mammary sign of IGM.
A 36-year-old nulliparous woman presented with fever and skin rash at our dermatology clinic at the North Franche-Comté hospital in July 2017. Symptoms had occurred 2 days previously. The patient had a fever of 38.8°C and typical erythema nodosum on the limbs. Physical examination showed an irregular, inflammatory, abscessed mass measuring 20cm with multiple shrinkage zones that had been developing for 4 weeks and for which breast examinations were being performed. Laboratory tests showed an inflammatory state (CRP 155mg/mL, WBC 14.6×109/L), other tests (serum electrolytes and calcium, hepatic and renal tests, Streptotest, T-spot, HIV, HBV, HCV serology, anti-streptolysin, anti-streptodornase, local microbiology samples, antinuclear antibodies, soluble antigens antibodies, hemocultures, angiotensin-converting-enzyme and chest x-rays) were normal. Microbiology investigations were negative. Mammography revealed invasive mastitis. Breast sample biopsies showed giant cell granulomas without caseous necrosis or tumor cells, and histochemical staining (PAS, Ziehl, Grocott, Gram) was negative. The final diagnosis was of IGM associated with erythema nodosum. Symptoms rapidly improved with oral steroids.
As reported herein, erythema nodosum may be associated with IGM and support the diagnosis thereof. This association is rare, with fewer than 30 case reports described in the literature. IGM is an uncommon benign disorder of the breast that can mimic two frequent breast disorders: breast carcinoma and breast abscess. It usually occurs in young sexually active women. It appears as a tumor with an inflammatory solid painful mass, with nipple and skin retraction, occasionally with abscesses and accompanied by homolateral axillary lymphadenopathies. Radiologic findings are usually not specific for or suggestive of cancer. The histopathological picture of IGM is characterized by the presence of multinucleated giant cells and epithelioid histiocytes forming non-caseating granulomas around lobules. Minor ductal and periductal inflammation is usually present. IGM is rarely associated with autoimmune system manifestations, especially erythema nodosum, arthritis, episcleritis or hidradenitis suppurativa. Although the physiopathology of IGM remains unclear, this case serves as additional evidence that the etiology of IGM is of autoimmune origin. While there is no recommendation for the treatment of IGM, oral steroids remain the cornerstone of therapy.
We report a case of IGM associated with erythema nodosum. Dermatologists must be aware of this association.
特发性肉芽肿性乳腺炎（IGM）是一种罕见的慢性肉芽肿性炎症性疾病，具有可能产生误导的临床和放射学特征。IGM是在排除感染性或肿瘤性疾病后做出的诊断。在此，我们报告一例与IGM相关的结节性红斑病例，这提醒我们结节性红斑可能是IGM的一种乳腺外表现。
一名36岁未育女性于2017年7月在北弗朗什 - 孔泰医院皮肤科门诊就诊，主诉发热和皮疹。症状于两天前出现。患者体温38.8°C，四肢有典型的结节性红斑。体格检查发现一个不规则的、炎症性的、脓肿性肿块，大小为20cm，有多个收缩区，已发展4周，正在进行乳房检查。实验室检查显示有炎症状态（CRP 155mg/mL，白细胞14.6×10⁹/L），其他检查（血清电解质和钙、肝肾功能检查、链球菌试验、T-spot、HIV、HBV、HCV血清学、抗链球菌溶血素、抗链道酶、局部微生物样本、抗核抗体、可溶性抗原抗体、血培养、血管紧张素转换酶和胸部X光）均正常。微生物学检查为阴性。乳房X线摄影显示为浸润性乳腺炎。乳房样本活检显示巨细胞肉芽肿，无干酪样坏死或肿瘤细胞，组织化学染色（PAS、齐尔、格罗科特、革兰氏）为阴性。最终诊断为与结节性红斑相关的IGM。口服类固醇后症状迅速改善。
如本文所报道，结节性红斑可能与IGM相关并支持其诊断。这种关联很罕见，文献中描述的病例报告少于30例。IGM是一种不常见的乳腺良性疾病，可模仿两种常见的乳腺疾病：乳腺癌和乳腺脓肿。它通常发生在年轻的性活跃女性中。表现为一个有炎症的实性疼痛肿块的肿瘤，伴有乳头和皮肤回缩，偶尔有脓肿，并伴有同侧腋窝淋巴结肿大。放射学表现通常对癌症无特异性或提示性。IGM的组织病理学特征是存在多核巨细胞和上皮样组织细胞，在小叶周围形成非干酪样肉芽肿。通常存在轻微的导管和导管周围炎症。IGM很少与自身免疫系统表现相关，尤其是结节性红斑、关节炎、巩膜外层炎或化脓性汗腺炎。虽然IGM的生理病理学仍不清楚，但该病例作为额外证据表明IGM的病因是自身免疫性的。虽然对于IGM的治疗没有推荐，但口服类固醇仍然是治疗的基石。
我们报告一例与结节性红斑相关的IGM病例。皮肤科医生必须意识到这种关联。
