Pemphigus 天疱疮

woman, otherwise healthy, in her 50’s, she was examined via online consultation by our clinic with a 3-month history of grouped blisters and erythematous plaques on her arms. She had received a diagnosis of pemphigus at local hospital. Laboratory studies showed a blood alkaline phosphatase level of 130 units per liter (reference range, 35 to 104 units per liter) and serum immunoglobulin A level of 4.530 gram per liter (reference range, 0.7000 to 4.0000 gram per liter). A Skin biopsy was not ordered or performed due to the COVID-19 lockdown by the local goverment. She was prescribed Dexamethasone (steroids) tablets and Transfer Factor supplement to relieve the symptoms, however her blisters did not subside.

Examination online through Zoom Apps showed elevated fluid-filled blisters and erosions on both her arms. The patient had no complain of ocular symptoms, hoarseness, dysphagia, dysuria, or dyspareunia. Mild pain and itchiness was reported. In this case, our herbal medicine Originax was initiated, thereafter the blisters and skin changes abated within 8 months. We thank patient for sending us the photos via WhatsApp and giving consent for them to be used for as testimonial.

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Discussion

The term pemphigus stems from the Greek ‘pemphix’, which means blister or bubble, and it describes a group of chronic blistering epithelial diseases in which the production of IgG autoantibodies against intercellular adhesion molecules of keratinocytes results in acantholysis (the loss of cell–cell adhesion between keratinocytes) [1-4]. Patients with pemphigus, autoimmune skin bullous disease, develop mucosal erosions and/or flaccid bullae, erosions, or pustules on skin. The four major entities of the pemphigus group include pemphigus vulgaris, pemphigus foliaceus, immunoglobulin A (IgA) pemphigus, and paraneoplastic pemphigus. The different forms of pemphigus are distinguished by their clinical features, associated autoantigens, and laboratory findings [2, 5-10].

pemphigus table

Follow-up Updated on 12 July 2022

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一位 50 多岁的其他方面健康女性到我们的诊所就诊,她有 3 个月的手臂分组水疱和红斑斑块病史。当地医院诊断为天疱疮。血液报告显示碱性磷酸酶水平为 130 单位/升(参考范围,35 至 104 单位/升),血清免疫球蛋白 A 水平为 4.530 克/升(参考范围,0.7000 至 4.0000 克/升)。因为冠状病毒封城的缘故,当地医院没有进行皮肤活检。处方地塞米松(类固醇)片剂和转移因子补充剂以缓解症状,但服药后水泡并未消退。

患者联络我诊所进行网上视频问诊,她提供的图片显示她的双臂上出现充满液体的水泡和糜烂。患者没有眼部症状、声音嘶哑、吞咽困难、排尿困难和性交困难,报告轻度疼痛和瘙痒。在这种情况下,我们开始使用我们的中药治疗,水泡和糜烂在 8 个月内得到缓解。我们感谢患者通过手机应用将照片发送给我们,并同意将其发表。

讨论

天疱疮一词源于希腊语“pemphix”,意为水疱或气泡,它描述了一组慢性水疱性上皮皮肤病,其中针对角质形成细胞的细胞间粘附分子的 IgG 自身抗体的产生导致棘层松解(细胞-细胞丢失角质形成细胞之间的粘附)[1-4]。患有天疱疮,这种自身免疫性皮肤大疱病,的患者会出现黏膜糜烂和/或松弛性大疱、糜烂或皮肤脓疱。天疱疮有四种类型;寻常天疱疮、落叶状天疱疮、免疫球蛋白 A (IgA) 天疱疮和副肿瘤性天疱疮。不同形式的天疱疮通过其临床特征、相关自身抗原和实验室检查结果来区分 [2, 5-10]。

Reference:

  1. Amagai, M. in Dermatology Vol. 1 Ch. 29, eds Bolognia, J. L., Jorizzo, J. L. & Schaffer, J. V.) 461–474 Mosby, 2012.
  2. Mihai S, Sitaru C. Immunopathology and molecular diagnosis of autoimmune bullous diseases. J Cell Mol Med 2007; 11:462.
  3. Sitaru C, Zillikens D. Mechanisms of blister induction by autoantibodies. Exp Dermatol 2005; 14:861.
  4. Hertl M, Eming R, Veldman C. T cell control in autoimmune bullous skin disorders. J Clin Invest 2006; 116:1159.
  5. Stanley JR, Amagai M. Pemphigus, bullous impetigo, and the staphylococcal scalded-skin syndrome. N Engl J Med 2006; 355:1800.
  6. Hashimoto T, Teye K, Ishii N. Clinical and immunological studies of 49 cases of various types of intercellular IgA dermatosis and 13 cases of classical subcorneal pustular dermatosis examined at Kurume University. Br J Dermatol 2017; 176:168.
  7. Prost C, Intrator L, Wechsler J, et al. IgA autoantibodies bind to pemphigus vulgaris antigen in a case of intraepidermal neutrophilic IgA dermatosis. J Am Acad Dermatol 1991; 25:846.
  8. Wang J, Kwon J, Ding X, et al. Nonsecretory IgA1 autoantibodies targeting desmosomal component desmoglein 3 in intraepidermal neutrophilic IgA dermatosis. Am J Pathol 1997; 150:1901.
  9. Kárpáti S, Amagai M, Liu WL, et al. Identification of desmoglein 1 as autoantigen in a patient with intraepidermal neutrophilic IgA dermatosis type of IgA pemphigus. Exp Dermatol 2000; 9:224.
  10. Ishii N, Ishida-Yamamoto A, Hashimoto T. Immunolocalization of target autoantigens in IgA pemphigus. Clin Exp Dermatol 2004; 29:62.