Successful treatment of linear IgA bullous dermatosis with mycophenolate mofetil.
Successful treatment of linear IgA bullous dermatosis with mycophenolate mofetil.
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吗替麦考酚酯成功治疗线性 IgA 大疱性皮肤病。
DOI：
10.1080/000155502320323351
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发表时间：
2002
影响因子：
3.6
通讯作者：
Michael Sticherlin
中科院分区：
文献类型：
--
作者：
R. Gläser;Michael Sticherlin
先生，线状伊加大疱性皮肤病（LABD）是一种罕见的获得性自身免疫性表皮下水疱性疾病，结合了疱疹样皮炎（DH）和大疱性类天疱疮（BP）的临床特征（1）。儿童慢性大疱病被认为是LABD的一种变异。两者之间唯一明显的区别是发病年龄较小（2）。LABD的特征在于伊加在基底膜区（BMZ）的线性沉积，并且在某些情况下通过循环伊加抗BMZ抗体。LABD和儿童期慢性大疱性疾病通常对图1有反应。首次出现时，使用砜类和葡萄糖皮质激素皮肤病腹部皮肤联合治疗的线性伊加大疱患者的临床特征：紧张的皮质类固醇，但在某些患者中可观察到替代治疗性大疱和糜烂。必须考虑各种办法。我们描述了一名LABD患者，该患者对重组BP 180-NC 16 A、ANA、抗ENA、抗口服霉酚酸酯（MMF）、一种免疫抑制剂或肌内膜抗体有反应，该药物也已成功用于大疱性二氨基二苯基类天疱疮的标准全身治疗（3-4）。单用DADPS或与糖皮质激素合用达0.5mg·kg ~（-1）体重，仅暂时有效。因此，启动了病例报告中的MMF治疗，随后临床改善。A 1998年2月，一名20岁的男性受试者在我诊所接受5个月的治疗后复发，主诉瘙痒性皮肤病变，通过在双肘周围增加高剂量，成功抑制了MMF。随后，病变播散剂量静脉注射免疫球蛋白（IVIG）在躯干和四肢，形成3次，间隔6周。紧张性大疱的详细治疗。我们的患者没有其他疾病的病史，时间表见表I。并呈现健康状态，无胃肠道症状自1999年5月以来，皮肤状况已稳定，症状或全身用药。在连续治疗下，用2g第1天的MMF作为躯干和伸肌表面的连续单次治疗。皮损愈合缓慢，局部偶见荨麻疹性丘疹和斑块残留，色素沉着。最初，含透明或出血性囊泡的MMF剂量降低导致病情加重。最终，系统进展为糜烂伴出血性结痂，可停止temic治疗，无恶化（图1）。在近一年半的总随访期内，所有常规血液学和化学实验室检查。参数在正常范围内。病变的腹部皮肤组织学检查讨论显示表皮下大疱充满水肿性体液、淋巴细胞、嗜酸性粒细胞和大量的嗜酸性粒细胞。我们的病人的临床、组织学和血清学特征是诊断LABD的。嗜中性粒细胞的检测。在真皮内发现了一种混合的渗透剂。用人盐裂皮肤作循环伊加抗体检测，见表皮线状染色，可见透明层。然而，在LABD中发现的伊加抗体的抗原特异性似乎是沿着BMZ的异源C3沿着。患者血清间接免疫荧光染色阳性，滴度为均匀。最近的结果表明，97-kD蛋白（97-LAD），这是最初确定的艾德内1：40在猴食管与伊加抗基底膜抗体和人分裂皮肤（1 mol/l的基底膜区的透明层代表的细胞外结构域的180 kD大疱氯化钠）显示在水泡顶部的线性染色模式。对人类天疱疮抗原（BPAg 2、BP 180）无特异性IgA反应性（5）。
Sir, Linear IgA bullous dermatosis (LABD) is a rare, acquired, autoimmune, subepidermal blistering disorder combining the clinical features characteristic of dermatitis herpetiformis (DH) and bullous pemphigoid (BP) (1). Chronic bullous disease of childhood is believed to be a variant of LABD. The only apparent diVerence between the two is the younger age at presentation (2). LABD is characterized by linear deposition of IgA at the basement membrane zone (BMZ) and in some cases by circulating IgA anti-BMZ-antibodies. LABD and chronic bullous disease of childhood generally respond Fig. 1. Clinical features of the patient with linear IgA bullous to a combined treatment with sulphones and glucocortidermatosis abdominal skin at the time of rst presentation: a tense costeroids, but in some patients alternative therapeutic bulla and erosions are seen. approaches have to be considered. We describe a patient with LABD who responded to recombinant BP180-NC16A, ANA, anti-ENA, antioral mycophenolate mofetil (MMF), a immunosuppresgliadineor endomysium antibodies could be found. sive drug that has also been used successfully in bullous Standard systemic therapy with diamino-diphenylpemphigoid (3–4). sulphone (DADPS) alone or in combination with glucocorticosteroids up to 0.5mgkg 1 body weight was only temporarily eVective. Therapy with MMF was therefore CASE REPORT initiated, with subsequent clinical improvement. A In February 1998, a 20-year-old male subject presented relapse that occurred after 5 months of therapy with himself at our clinic complaining of itching skin lesions MMF was successfully suppressed by additional higharound both elbows. Later, the lesions disseminated dose intravenous immunoglobulins (IVIG) administered over the trunk and the extremities, with formation of 3 times at intervals of 6 weeks. The detailed treatment tense bullae. Our patient had no history of other diseases schedule is listed in Table I. and presented in a healthy state, with no gastrointestinal Since May 1999 the skin condition has been stabilized symptoms or systemic medication. under continuing therapy with 2 g day 1 MMF as a On the trunk and the extensor surfaces, erythematous single treatment. The lesions healed slowly, with residual urticarial papules and plaques were occasionally covered local hyperpigmentation. Initially, a reduction of the with vesicles containing a clear or haemorrhagic uid MMF dosage resulted in exacerbation. Eventually, systhat progressed to erosions with haemorrhagic crusts temic therapy could be stopped, with no deterioration (Fig. 1). during a total follow-up period of nearly one and a All routine haematological and chemical laboratory half years. parameters were within the normal range. Histological examination of lesional abdominal skin DISCUSSION showed a subepidermal bulla lled with oedema uid, brin, lymphocytes, neutrophilic and numerous eosinoThe clinical, histological as well as serological features in our patient are diagnostic of LABD. The detection philic granulocytes. A mixed in ammatory in ltrate was found within the dermis. of circulating IgA antibodies with epidermal linear staining on human salt-split skin revealed the lamina-lucida Direct immuno uorescence examination of perilesional skin showed linear deposition of IgA, IgM and type of the disease. However, the antigen speci city of the IgA antibodies found in LABD seems to be heteroC3 along the BMZ. Indirect immuno uorescence staining with the patient’s serum was positive at a titre of geneous. Recent results indicate that the 97-kD-protein (97-LAD), which was initially identi ed within the 1:40 on monkey oesophagus with IgA anti-basement membrane antibodies and on human split skin (1mol/l lamina lucida of the basement membrane zone represents the extracellular domain of the 180 kD bullous sodium chloride) showing a linear staining pattern at the blister top. No speci c IgA-reactivity against human pemphigoid antigen (BPAg2, BP180) (5).