HS 治疗取决于疾病严重程度。Hurley 分类量表近似于就诊时疾病的严重程度,可用于将患者分层,接受越来越高强度的治疗。它有助于指导治疗方案的选择。[4]Hurley H. Axillary hyperhidrosis, apocrine bromhidrosis, hidradenitis suppurativa, and familial benign pemphigus. In: Roenigk RH, Roenigk HH Jr, eds. Dermatologic surgery: principles and practice. New York, NY: Marcel Dekker; 1989:729-739.[14]Martorell A, García FJ, Jiménez-Gallo D, et al. Update on hidradenitis suppurativa (part II): treatment. Actas Dermosifiliogr. 2015;106:716-724.http://www.actasdermo.org/en/update-on-hidradenitis-suppurative-part/articulo/S1578219015002449/http://www.ncbi.nlm.nih.gov/pubmed/26277040?tool=bestpractice.com
Hurley I 级(轻度):表现为脓肿和炎症结节但无瘢痕形成。
[Figure caption and citation for the preceding image starts]: I 级化脓性汗腺炎:发炎结节和丘疹不连续,其间有正常皮肤且无瘢痕形成由 R.A.Lee, MD, PhD 提供 [Citation ends].
Hurley II 级(中度):表现为脓肿和炎症结节并有瘢痕形成。但是,炎症皮损和瘢痕之间有正常皮肤区域分隔。
[Figure caption and citation for the preceding image starts]: II 级化脓性汗腺炎:发炎结节和瘢痕,其间有正常皮肤区域由 R.A.Lee, MD, PhD 提供 [Citation ends].
Hurley III 级(重度):瘢痕广泛相连,伴或不伴活跃性炎症皮损。
[Figure caption and citation for the preceding image starts]: III 级化脓性汗腺炎:瘢痕、囊肿、粉刺和炎症结节相连由 R.A.Lee, MD, PhD 提供 [Citation ends].
治疗通常涉及多学科。[1]Jemec GB. Clinical practice. Hidradenitis suppurativa. N Engl J Med. 2012;366:158-164.http://www.ncbi.nlm.nih.gov/pubmed/22236226?tool=bestpractice.com应考虑尽早转诊至相应专科(皮肤科、传染科或整形外科),以讨论医疗和手术方案。内科治疗分为 4 大类:抗生素、非甾体抗炎药 (NSAID)、激素制剂和全身用维 A 酸。虽然所有类别的药物均可在任何阶段使用,但是应考虑发生严重药物不良反应的可能性,并与疾病严重程度相平衡。
急性症状
可使用切开引流来缓解因脓液累积造成的疼痛和肿胀。但是,该疗法不可避免地会导致复发。也可口服抗生素,但可能需用药 6-8 周。
I 级(轻度)疾病
I 级定义为有 1 处或更多处脓肿,无窦道或瘢痕形成。[4]Hurley H. Axillary hyperhidrosis, apocrine bromhidrosis, hidradenitis suppurativa, and familial benign pemphigus. In: Roenigk RH, Roenigk HH Jr, eds. Dermatologic surgery: principles and practice. New York, NY: Marcel Dekker; 1989:729-739.
[Figure caption and citation for the preceding image starts]: I 级化脓性汗腺炎:发炎结节和丘疹不连续,其间有正常皮肤且无瘢痕形成由 R.A.Lee, MD, PhD 提供 [Citation ends].
应强调外用药。[14]Martorell A, García FJ, Jiménez-Gallo D, et al. Update on hidradenitis suppurativa (part II): treatment. Actas Dermosifiliogr. 2015;106:716-724.http://www.actasdermo.org/en/update-on-hidradenitis-suppurative-part/articulo/S1578219015002449/http://www.ncbi.nlm.nih.gov/pubmed/26277040?tool=bestpractice.com可定期局部用抗生素(如克林霉素或甲硝唑)或局部用抗菌素(如六氯酚或氯己定)以减少细菌过度生长。在一项临床试验中,局部用克林霉素 1% 溶液(一天 2 次)与口服四环素同样有效。[15]Jemec GB, Wendelboe P. Topical clindamycin versus systemic tetracycline in the treatment of hidradenitis suppurativa. J Am Acad Dermatol. 1998;39:971-974.http://www.ncbi.nlm.nih.gov/pubmed/9843011?tool=bestpractice.com局部治疗应持续 8 周。
选择局部还是全身用抗生素应基于细菌培养的敏感性、疾病程度和患者依从性。定期使用四环素类抗生素可有效降低 HS 皮损感染。[15]Jemec GB, Wendelboe P. Topical clindamycin versus systemic tetracycline in the treatment of hidradenitis suppurativa. J Am Acad Dermatol. 1998;39:971-974.http://www.ncbi.nlm.nih.gov/pubmed/9843011?tool=bestpractice.com治疗应至少持续 8 周。
疼痛程度通常与炎症程度有关。因此,针对炎症的治疗通常能够有效减轻疼痛。在使用其他止痛药(如对乙酰氨基酚)之前,应先按需使用 NSAID。
II 级(中度)疾病
II 级定义为复发 1 次或多次,广泛存在孤立的有窦道和瘢痕的脓肿。[4]Hurley H. Axillary hyperhidrosis, apocrine bromhidrosis, hidradenitis suppurativa, and familial benign pemphigus. In: Roenigk RH, Roenigk HH Jr, eds. Dermatologic surgery: principles and practice. New York, NY: Marcel Dekker; 1989:729-739.[14]Martorell A, García FJ, Jiménez-Gallo D, et al. Update on hidradenitis suppurativa (part II): treatment. Actas Dermosifiliogr. 2015;106:716-724.http://www.actasdermo.org/en/update-on-hidradenitis-suppurative-part/articulo/S1578219015002449/http://www.ncbi.nlm.nih.gov/pubmed/26277040?tool=bestpractice.com
[Figure caption and citation for the preceding image starts]: II 级化脓性汗腺炎:发炎结节和瘢痕,其间有正常皮肤区域由 R.A.Lee, MD, PhD 提供 [Citation ends].
II 级疾病患者应更长时间使用全身性抗生素。四环素类抗生素使用最广,但也应考虑使用其他种类的药物(如克林霉素)联用利福平。[14]Martorell A, García FJ, Jiménez-Gallo D, et al. Update on hidradenitis suppurativa (part II): treatment. Actas Dermosifiliogr. 2015;106:716-724.http://www.actasdermo.org/en/update-on-hidradenitis-suppurative-part/articulo/S1578219015002449/http://www.ncbi.nlm.nih.gov/pubmed/26277040?tool=bestpractice.com[16]Gener G, Canoui-Poitrine F, Revuz JE, et al. Combination therapy with clindamycin and rifampicin for hidradenitis suppurativa: a series of 116 consecutive patients.
Dermatology. 2009;219:148-154.http://www.ncbi.nlm.nih.gov/pubmed/19590173?tool=bestpractice.com甲氧苄啶/磺胺甲噁唑可能会造成严重的皮肤反应,因此被视为二线疗法。克林霉素加利福平治疗应持续 10 周。多西环素和甲氧苄啶/磺胺甲噁唑治疗应持续 8 周。
除口服抗生素外,应持续局部用抗生素或局部用抗菌药。应考虑对有明确的经前发病女性使用至少 8 周的抗雄激素药物(如螺内酯)。[17]Sawers RS, Randall VA, Ebling FJ. Control of hidradenitis suppurativa in women using combined antiandrogen (cyproterone acetate) and oestrogen therapy. Br J Dermatol. 1986;115:269-274.http://www.ncbi.nlm.nih.gov/pubmed/2944535?tool=bestpractice.com[18]Mortimer PS, Dawber RP, Gales MA, et al. A double-blind controlled cross-over trial of cyproterone acetate in females with hidradenitis suppurativa. Br J Dermatol. 1986;115:266-268.http://www.ncbi.nlm.nih.gov/pubmed/2944534?tool=bestpractice.com螺内酯的使用应仅限于采用充分节育措施的女性。应考虑使用抗炎药(如氨苯砜),联合或不联合短期使用(3-4 周)泼尼松(泼尼松龙)。[19]Kaur MR, Lewis HM. Hidradenitis suppurativa treated with dapsone: a case series of five patients. J Dermatolog Treat. 2006;17:211-213.http://www.ncbi.nlm.nih.gov/pubmed/16971313?tool=bestpractice.com通常在使用氨苯砜 12 周时可见改善。可能需要无限期施药以维持病情缓解。伴发寻常痤疮的患者可考虑口服异维 A 酸。[20]Boer J, van Gemert MJ. Long-term results of isotretinoin in the treatment of 68 patients with hidradenitis suppurativa. J Am Acad Dermatol. 1999;40:73-76.http://www.ncbi.nlm.nih.gov/pubmed/9922015?tool=bestpractice.com异维 A 酸为致畸药物,女性在使用该药治疗前应进行妊娠试验。患者还需要咨询该药可能存在的副作用。口服异维 A 酸应至少持续 6 个月。
III 级(重度)疾病
III 级定义为多发性脓肿以及相互连接的窦道与瘢痕。[4]Hurley H. Axillary hyperhidrosis, apocrine bromhidrosis, hidradenitis suppurativa, and familial benign pemphigus. In: Roenigk RH, Roenigk HH Jr, eds. Dermatologic surgery: principles and practice. New York, NY: Marcel Dekker; 1989:729-739.
[Figure caption and citation for the preceding image starts]: III 级化脓性汗腺炎:瘢痕、囊肿、粉刺和炎症结节相连由 R.A.Lee, MD, PhD 提供 [Citation ends].
III 级疾病治疗策略与 II 级疾病治疗策略重叠。但是,可能需要更为积极的治疗。TNF-α 抑制剂(例如英夫利西单抗、依那西普和阿达木单抗)是强效抗炎药,已成功用于治疗一些病例,尤其是在联合全身性应用抗生素时。[14]Martorell A, García FJ, Jiménez-Gallo D, et al. Update on hidradenitis suppurativa (part II): treatment. Actas Dermosifiliogr. 2015;106:716-724.http://www.actasdermo.org/en/update-on-hidradenitis-suppurative-part/articulo/S1578219015002449/http://www.ncbi.nlm.nih.gov/pubmed/26277040?tool=bestpractice.com[21]Giamarellos-Bourboulis EJ, Pelekanou E, Antonopoulou A, et al. An open-label phase II study of the safety and efficacy of etanercept for the therapy of hidradenitis suppurativa. Br J Dermatol. 2008;158:567-572.http://www.ncbi.nlm.nih.gov/pubmed/18076705?tool=bestpractice.com[22]Fardet L, Dupuy A, Kerob D, et al. Infliximab for severe hidradenitis suppurativa: transient clinical efficacy in 7 consecutive patients. J Am Acad Dermatol. 2007;56:624-628.http://www.ncbi.nlm.nih.gov/pubmed/17240478?tool=bestpractice.com[23]Trent JT, Kerdel FA. Tumor necrosis factor alpha inhibitors for the treatment of dermatologic diseases. Dermatol Nurs. 2005;17:97-107.http://www.ncbi.nlm.nih.gov/pubmed/15916184?tool=bestpractice.com[24]Alhusayen R, Shear NH. Pharmacologic interventions for hidradenitis suppurativa: what does the evidence say? Am J Clin Dermatol. 2012;13:283-291.http://www.ncbi.nlm.nih.gov/pubmed/22676319?tool=bestpractice.com[25]Blok JL, van Hattem S, Jonkman MF, et al. Systemic therapy with immunosuppressive agents and retinoids in hidradenitis suppurativa: a systematic review. Br J Dermatol. 2013;168:243-252.http://www.ncbi.nlm.nih.gov/pubmed/23106519?tool=bestpractice.com[26]Ingram JR, Woo PN, Chua SL, et al. Interventions for hidradenitis suppurativa. Cochrane Database Syst Rev. 2015;(10):CD010081.http://onlinelibrary.wiley.com/doi/10.1002/14651858.CD010081.pub2/fullhttp://www.ncbi.nlm.nih.gov/pubmed/26443004?tool=bestpractice.com[27]National Institute for Health and Care Excellence. Adalimumab for treating moderate to severe hidradenitis suppurativa. June 2016. https://www.nice.org.uk/ (last accessed 22 July 2017).https://www.nice.org.uk/guidance/ta392[28]Kimball AB, Okun MM, Williams DA, et al. Two phase 3 trials of adalimumab for hidradenitis suppurativa. N Engl J Med. 2016;375:422-434.http://www.nejm.org/doi/full/10.1056/NEJMoa1504370#t=articlehttp://www.ncbi.nlm.nih.gov/pubmed/27518661?tool=bestpractice.com虽然不认为它是单纯HS的标准治疗,但在治疗相关合并症(如炎症性肠病)时常会使用这些药物。[21]Giamarellos-Bourboulis EJ, Pelekanou E, Antonopoulou A, et al. An open-label phase II study of the safety and efficacy of etanercept for the therapy of hidradenitis suppurativa. Br J Dermatol. 2008;158:567-572.http://www.ncbi.nlm.nih.gov/pubmed/18076705?tool=bestpractice.com[22]Fardet L, Dupuy A, Kerob D, et al. Infliximab for severe hidradenitis suppurativa: transient clinical efficacy in 7 consecutive patients. J Am Acad Dermatol. 2007;56:624-628.http://www.ncbi.nlm.nih.gov/pubmed/17240478?tool=bestpractice.com[23]Trent JT, Kerdel FA. Tumor necrosis factor alpha inhibitors for the treatment of dermatologic diseases. Dermatol Nurs. 2005;17:97-107.http://www.ncbi.nlm.nih.gov/pubmed/15916184?tool=bestpractice.com[29]Grant A, Gonzalez T, Montgomery MO, et al. Infliximab therapy for patients with moderate to severe hidradenitis suppurativa: a randomized, double-blind, placebo-controlled crossover trial. J Am Acad Dermatol. 2010;62:205-217.http://www.ncbi.nlm.nih.gov/pubmed/20115947?tool=bestpractice.com应持续治疗至少 12 周,再在此时评估治疗效力。一般而言,可考虑联合用药来治疗重度疾病。泛发性瘢痕形成只能通过外科手术治疗。
瘢痕形成的外科管理
局部切除术适合边界清晰,并且较小的静态皮损。手术治疗的标准是切缘宽而深的广泛切除术。考虑到切除的大小和相应修复术以及已有特定激光机,可以转诊整形外科和/或皮肤外科。[30]van der Zee HH, Prens EP, Boer J. Deroofing: a tissue-saving surgical technique for the treatment of mild to moderate hidradenitis suppurativa lesions. J Am Acad Dermatol. 2010;63:475-480.http://www.ncbi.nlm.nih.gov/pubmed/20708472?tool=bestpractice.com[31]Tierney E, Mahmoud BH, Hexsel C, et al. Randomized control trial for the treatment of hidradenitis suppurativa with a neodymium-doped yttrium aluminium garnet laser. Dermatol Surg. 2009;35:1188-1198.http://www.ncbi.nlm.nih.gov/pubmed/19438670?tool=bestpractice.com[32]Mahmoud BH, Tierney E, Hexsel CL, et al. Prospective controlled clinical and histopathologic study of hidradenitis suppurativa treated with the long-pulsed neodymium:yttrium-aluminium-garnet laser. J Am Acad Dermatol. 2010;62:637-645.http://www.ncbi.nlm.nih.gov/pubmed/20227579?tool=bestpractice.com[33]Hazen PG, Hazen BP. Hidradenitis suppurativa: successful treatment using carbon dioxide laser excision and marsupialization. Dermatol Surg. 2010;36:208-213.http://www.ncbi.nlm.nih.gov/pubmed/20039918?tool=bestpractice.com
一般不建议采用破坏性方法,如冷冻疗法。二氧化碳激光机和 Nd-YAG 激光机的消融治疗显示有前景。对单独表皮化窦道选择性使用“去顶术”可有效治疗特异性复发性皮损。