中醫皮膚科專題--類天疱瘡

中藥內服及外用藥膏合併西醫治療類天疱瘡臨床個案分享

任之堂中醫診所鄭名惠醫師

       類天疱瘡（Bullous pemphigoid, BP）是自體免疫性疾病，其特徵為因表皮與真皮交界處的被自體免疫攻擊而分離導致出現大水泡，以及搔癢疼痛感。這類自體免疫疾病可能與以下有關：外傷或手術、燒傷、放射線治療、紫外線照射；也可能與某些藥物有關，例如：利尿劑(loop diuretics)、醛固酮拮抗劑(aldosterone antagonists)、糖尿病藥物(dipeptidyl peptidase-4 inhibitors, DPP-4）、癌症免疫療法藥物(programmed death-1 and programmed death ligand-1 inhibitors)、以及抗生素(penicillin)；此外，某些神經系統疾病亦可能有相關聯。類天疱瘡的病程可能是會在數月至數年內程度自然緩解，也可能呈現慢性反覆發作狀態。診斷的方式包括組織切片的病理檢查、直接或間接免疫螢光檢查，以及酵素免疫分析（ELISA）檢測。

       類天疱瘡的西醫第一線治療通常是強效外用類固醇。若無效，可改用口服類固醇( prednisone）或靜脈注射脈衝類固醇。若患者不適合使用口服類固醇，則可改用口服四環素類抗生素以控制病情並預防感染。若患者對類固醇或抗生素產生副作用，則可考慮使用免疫抑制劑，如azathioprine, dapsone, dupilumab,methotrexate, or mycophenolate mofetil。然而，長期使用高劑量類固醇與免疫抑制劑常導致顯著副作用。

       類天疱瘡在亞洲的發生率不高，僅為每百萬人中有5.6例，其三年內死亡率卻可能高達40%，與敗血症、肺炎、泌尿道感染等併發症及藥物副作用相關。近來多次在長期服用多重慢性病藥物病患者身上看見類天袍瘡的發生。

        這些患者因為已經多重用藥，這時候再增加治療類天疱瘡的藥物，除了增加多重藥物交互作用風險，也因為這類患者體能恢復力多不佳，所以經常病情反覆纏綿，越加虛弱。若是急性全身大發作患者，常體無完膚，換藥疼痛受剝皮之苦，恢復速度緩慢，反覆發燒感染，這時中西醫合併治療就能調節患者自體免疫過度亢進，減少水泡增生，縮短病程恢復更快。

         2023年12月底，我們中醫協助中西醫整合醫療，照顧一位60歲女性患者，有糖尿病、高血壓與高血脂病史，長期規律服用Exforge、Trajenta Duo與Lipitor等藥物多年。因為水泡、皮膚糜爛與潰瘍持續一個月，伴隨嚴重搔癢感。經病理組織檢查後確診為水泡性類天疱瘡，於2023年12月26日住進隔離病房。

         患者在住院11天內接受多種藥物治療，包括免疫調節劑、奎寧、抗組織胺藥、注射與口服類固醇、止痛藥、抗生素、Sabina乳膏及抗生素藥膏，但水泡、糜爛與潰瘍的症狀仍然嚴重持續。經會診中醫師，自2024年1月5日至2024年2月23日，開始為患者開立中藥內服藥物調整免疫與中藥物外用膏劑治療，盈顯發現中藥能加速傷口癒合、減少免疫發炎形成水泡和皮膚發炎潰爛狀態。

         也經中藥學機轉文章發現一些中藥，例如：柴胡劑、地黃、清熱解毒藥(黃芩、虎杖等)可調節減緩免疫發炎過程，利濕藥(蒼朮、爐甘石等)可減少水泡產生，選用蜜蠟阿膠等可以外用皮膚修復等。

整個病程治療過程已投稿EXPLORE期刊(impact factor : 2.2)，詳細文章內容可參閱網站https://www.sciencedirect.com/science/article/abs/pii/S1550830725000230?via%3Dihub

中西醫合併治療重症，雙重照護縮短病程。詳情請諮詢任之堂中醫診所醫師。

參考文章

1. Schmidt E, Zillikens D. Pemphigoid diseases. Lancet. 2013;381(9863):320–332.

 2. Venning VA, et al. British Association of Dermatologists’ guidelines for the

management of bullous pemphigoid 2012. Br J Dermatol. 2012;167(6):1200–1214.

 3. Montagnon CM, et al. Subepithelial autoimmune blistering dermatoses: clinical

features and diagnosis. J Am Acad Dermatol. 2021;85(1):1–14.

 4. Borradori L, et al. Updated S2 K guidelines for the management of bullous

pemphigoid initiated by the European Academy of Dermatology and Venereology

(EADV). J Eur Acad Dermatol Venereol. 2022;36(10):1689–1704.

 5. Singh S, et al. Interventions for bullous pemphigoid. Cochrane Database Syst Rev.

2023;8(8), Cd002292.

 6. Patel PM, et al. A Review Comparing International Guidelines for the Management

of Bullous Pemphigoid, Pemphigoid Gestationis, Mucous Membrane Pemphigoid,

and Epidermolysis Bullosa Acquisita. Am J Clin Dermatol. 2020;21(4):557–565.

 7. Persson MSM, et al. The global incidence of bullous pemphigoid: a systematic review

and meta-analysis. Br J Dermatol. 2022;186(3):414–425.

 8. Chung JG, et al. Bullous pemphigoid: its incidence, mortality and clinical outcome in

New Zealand. Australas J Dermatol. 2022;63(2):197–203.

 9. Ren Z, et al. Hospitalization, inpatient burden and comorbidities associated with

bullous pemphigoid in the U.S.A. Br J Dermatol. 2017;176(1):87–99.

 10. Hsieh SC, et al. The prescribing of Chinese herbal products in Taiwan: a cross-

sectional analysis of the national health insurance reimbursement database.

Pharmacoepidemiol Drug Saf. 2008;17(6):609–619.

 11. Liu BG, Li ZY, Du M. [Effects of jingui shenqi pill combined prednisone on

expression of glucocorticoid receptor and its clinical effect in treating bullous

pemphigoid patients]. Zhongguo Zhong Xi Yi Jie He Za Zhi. 2006;26(10):881–884.

 12. Baroni A, et al. The Use of Traditional Chinese Medicine in Some Dermatologic

Diseases: part II–Autoimmune Bullous Disorders and Lichen Planus. Skinmed. 2015;

13(3):195–203. quiz 204.

 13. Ho TJ, et al. The curative effects of the traditional Chinese herbal medicine

"Jinchuang ointment" on excisional wounds. Chin Med. 2020;15:41.

 14. Li Q, et al. Effects of the Chinese Herb Medicine Formula "She-Xiang-Yu-Hong"

Ointment on Wound Healing Promotion in Diabetic Mice. Evid Based Complement

Alternat Med. 2022;2022, 1062261.

 15. Wang J, et al. Clinical application of traditional Chinese medicine moisture exposed

burn ointment in the treatment of facial soft tissue defect. J Cosmet Dermatol. 2022;

21(6):2481–2487.

 16. Cheng T-H, et al. A biomimetic micropillar wound dressing with flavone and

polyphenol control release in vitro and in vivo. J Taiwan Inst Chem Eng. 2024,

105385.

 17. Lin E-T, et al. Efficient fucoidan extraction and purification from Sargassum

cristaefolium and preclinical dermal biological activity assessments of the purified

fucoidans. J Taiwan Inst Chem Eng. 2022;137, 104294.

 18. Wu PY, et al. Complementary Chinese Herbal Medicine Therapy Improves Survival

in Patients With Pemphigus: a Retrospective Study From a Taiwan-Based Registry.

Front Pharmacol. 2020;11, 594486.

 19. Ge XL, et al. Treatment of bullous pemphigoid in Chinese patients with Tripterygium

wilfordii Hook F. Dermatol Ther. 2020;33(4), e13721.

 20. XZ W. Clin Observ Curative Effect Pemphigus Treated Comp Glycyrrhizin Tablet. 2006;8:

61–62.

 21. Ma HD, et al. Traditional Chinese medicine and immune regulation. Clin Rev Allergy

Immunol. 2013;44(3):229–241.

 22. Matsumoto T, Shibata T. The ex vivo effect of the herbal medicine sho-saiko-to on

histamine release from rat mast cells. Eur Arch Otorhinolaryngol. 1998;255(7):

359–364.

 23. Bailly C. Efficacy and safety of the traditional herbal medication Chai-Ling-Tang (in

China), Siryung-tang (in Republic of Korea) or Sairei-To (in Japan).

J Ethnopharmacol. 2024;319(Pt 1), 117127.

 24. Tran NKS, et al. Multitargeted herbal prescription So Shiho Tang: a scoping review

on biomarkers for the evaluation of therapeutic effects. Pharmaceuticals (Basel).

2023;16(10):2371.

 25. Jia J, et al. Progress of research into the pharmacological effect and clinical

application of the traditional Chinese medicine Rehmanniae Radix. Biomed

Pharmacother. 2023;168, 115809.

 26. Xiao L, et al. Enzyme-digested Colla Corii Asini (E’jiao) accelerates wound healing

and prevents ultraviolet A-induced collagen synthesis decline and wrinkle formation

in three-dimensional skin equivalents. Hum Cell. 2020;33(4):1056–1067.

 27. Xiao L, et al. Enzyme-digested Colla Corii Asini (E’jiao) accelerates wound healing

and prevents ultraviolet A-induced collagen synthesis decline and wrinkle formation

in three-dimensional skin equivalents. Hum Cell. 2020;33(4):1056–1067.

 28. Zuo ZHAO, Z L, Jinming LI, Zhikun TANG. Research progress in traditional Chinese

medicine treatment of Pemphigus Vulgaris. J Emerg Trad Chin Med. 2021;30(2):

373–376.

 29. Nong Y, et al. A review of the use of beeswax in skincare. J Cosmet Dermatol. 2023;22

(8):2166–2173.

 30. Stohs SJ, Bagchi D. Antioxidant, Anti-inflammatory, and Chemoprotective

properties of acacia catechu heartwood extracts. Phytother Res. 2015;29(6):818–824.

 31. Jyoti, Garg V. Acacia catechu Willd. and Acacia arabica Willd. decrease the extent of

anxiety behavior by reducing oxidative stress and moderating neurochemicals.

J Ethnopharmacol. 2023;312, 116496.

 32. Naganuma M. Treatment with indigo naturalis for inflammatory bowel disease and

other immune diseases. Immunol Med. 2019;42(1):16–21.

 33. Zhang Q, et al. Psoriasis treatment using Indigo Naturalis: progress and strategy.

J Ethnopharmacol. 2022;297, 115522.

 34. Lee CL, et al. An alkaloid-rich phytopharmaceutical prepared from Qing Dai against

IL-17A-induced psoriasis. J Ethnopharmacol. 2024;318(Pt A), 116924.

 35. Lee CL, et al. IL-17A inhibitions of indole alkaloids from traditional Chinese

medicine Qing Dai. J Ethnopharmacol. 2020;255, 112772.

 36. Ruifeng W. Topical application of talcum powder. J Ext Therapy Trad Chin Med.

1999;8:35.

 37. Xingang Z. Observation on the therapeutic effect of Chinese medicine Mangxiao in

the treatment of acute eczema. China Pract Med. 2016;11:176–177.

 38. Ru JIA, J C-g, Ming YANG, Wei WANG. Review on the processing and applications

of calamine. Res Pract Chin Med. 2020;34:74–78.

 39. Wang S, et al. A clinical and mechanistic study of topical borneol-induced analgesia.

EMBO Mol Med. 2017;9(6):802–815.