Sharif Zaina, Javadzadeh Shagayegh, Boissiere Jeanne, Creamer Daniel
Department of Dermatology King's College Hospital NHS Foundation Trust London UK.
Skin Health Dis. 2024 Jun 3;4(4):e393. doi: 10.1002/ski2.393. eCollection 2024 Aug.
A 47 year old Caucasian female with a background of type 2 diabetes mellitus, hypothyroidism, and autoimmune hepatitis presented with a painful, pruritic, papular eruption in a photosensitive distribution across the upper chest, neck, face, dorsal hands and forearms. On examination, lesions coalesced into annular plaques each with an active, raised margin and an atrophic, yellow centre. Histopathology demonstrated an absence of mucin and elastophagocytosis with giant cells engulfing dermal elastin fibres. These histopathological features favoured a diagnosis of annular elastolytic giant cell granuloma (AEGCG). The patient was managed ciclosporin monotherapy 125 mg twice daily (3 mg/kg/day). At 8 week review, there was a marked improvement in the physical appearance of the dermatosis as well as diminishing of symptoms such as itch and cutaneous pain. AEGCG is a rare inflammatory dermatosis typically affecting sun-exposed sites. It has been proposed that AEGCG is triggered by a solar induced elastolysis however other theories suggest it is a primary granulomatous disorder and not a photodermatosis. AEGCG appears to be aligned to an autoimmune diathesis, indicated by its frequent association with autoimmune conditions such as Hashimoto's thyroiditis, vitiligo, giant cell arteritis and, as in our patient, auto-immune hepatitis. Diabetes mellitus occurring concurrently with AEGCG has also been observed, again like our patient. Histopathological features which distinguish AEGCG from granuloma annulare include absent mucin, absent necrobiosis, giant cells with more nuclei, non-palisading granulomata and marked loss of elastic tissue. AEGCG is often unresponsive to standard therapies. The literature indicates varying responses to photo-protection, topical/systemic/intralesional corticosteroids, and oral medications such as methotrexate, hydroxychloroquine, and dapsone. Few case reports have also documented improvement with ciclosporin. In aggressive forms of AEGCG, as in our patient, treatment with ciclosporin may be an effective intervention and should be initiated early in the disease.
一名47岁的白种女性，有2型糖尿病、甲状腺功能减退和自身免疫性肝炎病史，其胸部上方、颈部、面部、手背和前臂出现了呈光敏性分布的疼痛性、瘙痒性丘疹疹。检查时，病变融合成环状斑块，每个斑块都有活跃的、隆起的边缘和萎缩的黄色中心。组织病理学显示无黏蛋白，无坏死性改变，有巨细胞吞噬真皮弹性纤维（弹力组织吞噬作用）。这些组织病理学特征支持环状弹性组织溶解性巨细胞肉芽肿（AEGCG）的诊断。该患者接受环孢素单药治疗，每日两次，每次125毫克（3毫克/千克/天）。在8周复查时，皮肤病的外观有明显改善，瘙痒和皮肤疼痛等症状也有所减轻。AEGCG是一种罕见的炎症性皮肤病，通常累及暴露于阳光下的部位。有人提出AEGCG是由日光诱导的弹性组织溶解引发的，然而其他理论认为它是一种原发性肉芽肿性疾病，而非光皮肤病。AEGCG似乎与自身免疫素质有关，这表现为它经常与自身免疫性疾病相关，如桥本甲状腺炎、白癜风、巨细胞动脉炎，以及像我们这位患者的自身免疫性肝炎。同时患有AEGCG和糖尿病也曾被观察到，同样如我们的患者。将AEGCG与环状肉芽肿区分开来的组织病理学特征包括无黏蛋白、无渐进性坏死、多核巨细胞、非栅栏状肉芽肿以及弹性组织的明显丧失。AEGCG通常对标准治疗无反应。文献表明对光防护、外用/系统/皮损内注射皮质类固醇以及口服药物如甲氨蝶呤、羟氯喹和氨苯砜有不同的反应。也有少数病例报告记录了使用环孢素后病情改善的情况。对于像我们患者这样的侵袭性AEGCG，使用环孢素治疗可能是一种有效的干预措施，应在疾病早期开始使用。
