Galli‐Galli disease responsive to isotretinoin treatment
Galli‐Galli disease responsive to isotretinoin treatment
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Galli-Galli 病对异维A酸治疗有反应
DOI：
10.1111/ijd.13975
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发表时间：
2018
影响因子：
3.6
通讯作者：
J. Hsiao
中科院分区：
文献类型：
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作者：
Elizabeth Dupuy;S. Alexanian;J. Hsiao
一名 58 岁女性有 15 年皮疹瘙痒病史，皮疹始于躯干，随后蔓延至四肢。她报告说过去曾进行过多次不确定的皮肤活检。阿维A此前曾帮助改善皮疹，但因脱发而停药。外用类固醇只能暂时缓解瘙痒。她指出，她的母亲也有类似的皮疹，但程度较轻，并在外部机构被诊断出患有加利-加利病。体格检查发现大量角化过度、红棕色、平顶丘疹，部分周围有痂皮，躯干和上下肢有雀斑样斑疹背景（图 1）。弯曲区域的病变、粉刺和色素减退病变没有加重。皮肤活检显示局灶性棘层松解性角化不良和覆盖细长网状脊的融合性角化不全。乳头上板变薄，局灶性基底色素沉着过度，浅表血管周围淋巴组织细胞浸润（图2）。根据这些临床和组织学发现，患者被诊断为 Galli-Galli 病。她推迟了基因检测。由于患者之前对阿维A有反应，我们讨论让她尝试异维A酸，看看是否有好处且不会产生无法忍受的副作用。患者开始使用异维A酸（30 毫克/天）和 0.1% 曲安西龙乳膏。经过仅仅 1 个月的治疗，她报告说她的皮疹得到了改善（红色、凸起和瘙痒减少），并且不需要类固醇霜。将异维A酸剂量增加至 40 毫克/天两周后，患者报告面部皮肤出现“起泡”反应，于是她停止了用药。目前还不清楚是什么原因导致了这种反应（以及是否与光敏性有关），因为她没有来接受评估。如果她的皮肤病再次发作，患者确实计划返回诊所重新开始服用低剂量异维A酸。图 1 临床表现。下肢多发角化过度、红棕色平顶丘疹，部分周围有鳞屑
A 58-year-old woman presented with a 15-year history of an itchy skin eruption that started on her trunk and subsequently spread to her extremities. She reported having had multiple inconclusive skin biopsies in the past. Acitretin had previously helped the rash improve but was discontinued due to hair loss. Topical steroids only temporarily relieved itching. She noted that her mother had a similar, though milder, skin eruption and had been diagnosed with Galli-Galli disease at an outside institution. Physical exam was notable for numerous hyperkeratotic, redbrown, flat-topped papules, some with overlying peripheral crust, with a background of lentigo-like macules on the trunk and upper and lower extremities (Fig. 1). Accentuation of lesions in flexural regions, comedones, and hypopigmented lesions were absent. A skin biopsy revealed focal acantholytic dyskeratosis and confluent parakeratosis overlying elongated rete ridges. There was thinning of the suprapapillary plate, focal basilar hyperpigmentation, and a superficial perivascular lymphohistiocytic infiltrate (Fig. 2). With these clinical and histologic findings, the patient was diagnosed with Galli-Galli disease. She deferred genetic testing. Since the patient had previously responded to acitretin, we discussed having her try isotretinoin to see if there would be a benefit without an intolerable side effect. The patient was started on isotretinoin (30 mg/day) and also triamcinolone 0.1% cream. After just 1 month of treatment, she reported that her rash was improved (less red, raised, and itchy), and she did not need the steroid cream. Two weeks after increasing her isotretinoin dose to 40 mg/day, the patient reported a “blistering” skin reaction on her face, and she stopped the medication. It is unclear what caused this reported reaction (and whether it was related to photosensitivity), as she did not come in for evaluation. The patient does plan to return to clinic to restart lowdose isotretinoin if her skin disease flares again. Figure 1 Clinical presentation. Multiple hyperkeratotic, red-brown flat-topped papules, some with peripheral scale, on the lower extremities