SAHA syndrome

SAHA syndrome is a cutaneous syndrome characterized by seborrhoea, acne, hirsutism and alopecia, first described in 1982 and frequently associated with hyperandrogenismSAHA 综合征是一种皮肤综合征，以脂溢性皮炎、痤疮、多毛症和脱发为特征，于1982年首次描述，常与高雄激素血症相关。

The acronym SAHA derives from the four cardinal cutaneous signs: Seborrhoea, Acne, Hirsutism and Alopecia缩写 SAHA 源自四种主要皮肤体征：脂溢性皮炎（Seborrhoea）、痤疮（Acne）、多毛症（Hirsutism）和脱发（Alopecia）。

The syndrome is also known as dermatological androgenization syndrome and is frequently associated with polyendocrine metabolic ovarian syndrome, cystic mastitis, obesity and infertility该综合征也称为皮肤雄激素化综合征，常与多内分泌代谢性卵巢综合征、囊性乳腺炎、肥胖和不孕症相关。

Terminology and classification

• SAHA syndrome is classified as a cutaneous condition with hyperandrogenic features, and is grouped with other androgen-dependent dermatoses such as the HAIR-AN syndromeSAHA 综合征归类为具有高雄激素特征的皮肤疾病，与其他雄激素依赖性皮肤病如 HAIR-AN 综合征同组。^[1]
• The condition is also known as dermatological androgenization syndrome, reflecting the cutaneous manifestation of systemic androgen excess该病也称为皮肤雄激素化综合征，反映了系统性雄激素过多的皮肤表现。^[1]
• SAHA syndrome overlaps clinically with other hyperandrogenic disorders including polycystic ovary syndrome, idiopathic hirsutism and androgen-secreting tumoursSAHA 综合征在临床上与其他高雄激素疾病重叠，包括多囊卵巢综合征、特发性多毛症和雄激素分泌性肿瘤。
• The syndrome is distinguished from the HAIR-AN syndrome (hyperandrogenism, insulin resistance, acanthosis nigricans) by the absence of insulin resistance and acanthosis nigricans该综合征通过缺乏胰岛素抵抗和黑棘皮病与 HAIR-AN 综合征（高雄激素血症、胰岛素抵抗、黑棘皮病）区分。
• No specific ICD code exists for SAHA syndrome, and it is usually classified under disorders of androgen excess or under the underlying endocrine disorderSAHA 综合征没有特定的 ICD 编码，通常归类于雄激素过多疾病或潜在内分泌疾病下。^[1]
• The syndrome is one of the cutaneous markers of hyperandrogenism, along with seborrhoea and hidradenitis suppurativa该综合征是高雄激素血症的皮肤标志之一，与脂溢性皮炎和化脓性汗腺炎并列。

History

• SAHA syndrome was first described in 1982 by Orfanos and colleagues, who recognized the clustering of four androgen-dependent cutaneous signsSAHA 综合征由 Orfanos 等于1982年首次描述，他们认识到四种雄激素依赖性皮肤体征的聚集。^[1]
• The acronym was introduced to standardize the description of the cutaneous signs of hyperandrogenism and to facilitate clinical recognition该缩写被引入以标准化高雄激素血症皮肤体征的描述并促进临床识别。^[1]
• Subsequent studies linked the cutaneous signs to underlying endocrine abnormalities, including ovarian and adrenal androgen excess后续研究将这些皮肤体征与潜在内分泌异常联系起来，包括卵巢和肾上腺雄激素过多。
• The association with polyendocrine metabolic ovarian syndrome was confirmed in the 1990s, establishing the syndrome as a cutaneous marker of endocrine disease与多内分泌代谢性卵巢综合征的关联在1990年代得到确认，确立了该综合征作为内分泌疾病皮肤标志的地位。
• Recent research has focused on the role of insulin resistance and the metabolic syndrome in the pathogenesis of androgen-dependent dermatoses最近的研究集中在胰岛素抵抗和代谢综合征在雄激素依赖性皮肤病发病机制中的作用。

Epidemiology

• SAHA syndrome is uncommon, and its exact prevalence is not well established because it is frequently under-recognized in dermatology practiceSAHA 综合征不常见，其确切患病率尚未充分确定，因为它在皮肤科实践中常被认识不足。^[1]
• The condition predominantly affects women of reproductive age, and is uncommon before puberty or after menopause该病主要影响育龄女性，青春期前或绝经后不常见。
• The prevalence of hyperandrogenism in women of reproductive age is approximately 5 to 10 per cent, with a subset of these women having SAHA syndrome育龄女性中高雄激素血症的患病率约为5%至10%，其中一部分女性患有 SAHA 综合征。
• Polycystic ovary syndrome, the most common cause of hyperandrogenism, affects approximately 1 in 10 women of reproductive age and is associated with SAHA syndrome in many cases多囊卵巢综合征是高雄激素血症最常见的原因，影响约十分之一的育龄女性，在许多病例中与 SAHA 综合征相关。
• Risk factors include obesity, insulin resistance, family history of hyperandrogenism and endocrine disorders affecting ovarian or adrenal androgen production危险因素包括肥胖、胰岛素抵抗、高雄激素血症家族史以及影响卵巢或肾上腺雄激素产生的内分泌疾病。

Pathophysiology

• SAHA syndrome arises from excess androgen production or increased androgen sensitivity in susceptible skin, particularly the pilosebaceous unitSAHA 综合征源于雄激素产生过多或易感皮肤（特别是毛皮脂腺单位）中雄激素敏感性增加。^[1]
• Androgens stimulate sebaceous gland activity, causing seborrhoea and acne, and promote terminal hair growth in androgen-dependent areas, causing hirsutism雄激素刺激皮脂腺活动，引起脂溢性皮炎和痤疮，并促进雄激素依赖区域的终毛生长，引起多毛症。^[1]
• Androgens also shorten the anagen phase of the hair cycle in genetically susceptible scalp follicles, leading to androgenetic alopecia雄激素还缩短遗传易感头皮毛囊中毛发生长周期的生长期，导致雄激素性脱发。
• The excess androgen may originate from the ovaries, the adrenal glands, or both, and can result from enzyme deficiencies in steroidogenesis过多的雄激素可能来源于卵巢、肾上腺或两者，并可由类固醇生成中的酶缺陷引起。
• Peripheral conversion of weaker androgens to more potent androgens in the skin amplifies the cutaneous effects皮肤中外周将较弱的雄激素转化为更强的雄激素会放大皮肤效应。
• Insulin resistance, when present, contributes to hyperandrogenism by increasing ovarian androgen production and reducing hepatic sex hormone-binding globulin synthesis当存在胰岛素抵抗时，通过增加卵巢雄激素产生和减少肝性激素结合球蛋白合成而促成高雄激素血症。

Clinical features

• The four cardinal features are seborrhoea (greasy skin), acne (particularly of the face and trunk), hirsutism (male-pattern terminal hair growth in women) and alopecia (androgenetic hair loss)四种主要特征是脂溢性皮炎（油性皮肤）、痤疮（特别是面部和躯干）、多毛症（女性男性型终毛生长）和脱发（雄激素性脱发）。^[1]
• Seborrhoea typically affects the face, scalp and upper trunk, and may precede the development of acne脂溢性皮炎通常影响面部、头皮和上躯干，可能先于痤疮的发展。^[1]
• Acne is often inflammatory and may be severe, with nodules and cysts in some patients痤疮常为炎症性，可能严重，部分患者出现结节和囊肿。^[1]
• Hirsutism affects androgen-dependent areas including the upper lip, chin, chest, abdomen and inner thighs多毛症影响雄激素依赖区域，包括上唇、下巴、胸部、腹部和大腿内侧。^[1]
• Alopecia typically shows a male-pattern distribution on the vertex and frontal scalp, with preservation of the frontal hairline in some women脱发通常表现为顶部和前额头皮的男性型分布，部分女性保留前发际线。
• Associated features may include menstrual irregularities, infertility, obesity, cystic mastitis and features of polycystic ovary syndrome相关特征可能包括月经不规律、不孕症、肥胖、囊性乳腺炎和多囊卵巢综合征的特征。

Diagnosis

• Diagnosis is clinical, based on the presence of the four cardinal cutaneous signs, and is supported by biochemical evidence of androgen excess诊断是临床性的，基于四种主要皮肤体征的存在，并得到雄激素过多生化证据的支持。^[1]
• Hormonal assessment includes measurement of total and free testosterone, dehydroepiandrosterone sulfate, androstenedione, luteinizing hormone and follicle-stimulating hormone激素评估包括测量总睾酮和游离睾酮、硫酸脱氢表雄酮、雄烯二酮、促黄体生成素和促卵泡激素。
• Elevated free testosterone or dehydroepiandrosterone sulfate suggests an underlying ovarian or adrenal source of androgen excess游离睾酮或硫酸脱氢表雄酮升高提示雄激素过多的潜在卵巢或肾上腺来源。
• Pelvic ultrasound is used to assess for polycystic ovary morphology, and adrenal imaging is indicated when an adrenal tumour is suspected盆腔超声用于评估多囊卵巢形态，当怀疑肾上腺肿瘤时指示肾上腺影像学检查。
• The differential diagnosis includes polycystic ovary syndrome, congenital adrenal hyperplasia, Cushing syndrome, androgen-secreting tumours and idiopathic hirsutism鉴别诊断包括多囊卵巢综合征、先天性肾上腺增生、库欣综合征、雄激素分泌性肿瘤和特发性多毛症。
• Ovarian and adrenal tumours should be excluded in patients with rapid onset of virilization or markedly elevated androgen levels在快速出现男性化或雄激素水平显著升高的患者中应排除卵巢和肾上腺肿瘤。

Management

• Management targets the underlying endocrine disorder and the individual cutaneous manifestations管理针对潜在内分泌疾病和个体皮肤表现。^[1]
• Combined oral contraceptives are first-line therapy for ovarian hyperandrogenism, reducing ovarian androgen production and increasing sex hormone-binding globulin复方口服避孕药是卵巢高雄激素血症的一线治疗，减少卵巢雄激素产生并增加性激素结合球蛋白。
• Anti-androgens such as spironolactone, cyproterone acetate and flutamide are used to block androgen action at the receptor level抗雄激素药物如螺内酯、醋酸环丙孕酮和氟他胺用于在受体水平阻断雄激素作用。
• Topical therapies for acne include retinoids, benzoyl peroxide and topical antibiotics, while topical minoxidil and anti-androgens are used for androgenetic alopecia痤疮的局部治疗包括维A酸类、过氧化苯甲酰和局部抗生素，而局部米诺地尔和抗雄激素用于雄激素性脱发。
• Metformin may be used in patients with insulin resistance, improving both metabolic parameters and hyperandrogenism二甲双胍可用于胰岛素抵抗患者，改善代谢参数和高雄激素血症。
• The prognosis depends on the underlying cause, with most patients achieving good control of cutaneous symptoms with appropriate endocrine and dermatological treatment预后取决于潜在原因，大多数患者通过适当的内分泌和皮肤病治疗可获得皮肤症状的良好控制。^[1]

Research and controversies

• The relationship between SAHA syndrome and polycystic ovary syndrome remains debated, with some authorities considering SAHA syndrome a cutaneous variant of PCOSSAHA 综合征与多囊卵巢综合征之间的关系仍有争议，一些权威认为 SAHA 综合征是 PCOS 的皮肤变异型。
• The role of insulin resistance in the absence of obesity is an area of active research, with implications for targeted therapy非肥胖情况下胰岛素抵抗的作用是活跃的研究领域，对靶向治疗有启示。
• There is no consensus on the optimal treatment sequence for the combination of acne, hirsutism and alopecia, and management is often individualized对于痤疮、多毛症和脱发的组合，最佳治疗顺序尚未达成共识，管理常个体化。
• Several clinical trials are registered on ClinicalTrials.gov for treatments of hyperandrogenism and polycystic ovary syndromeClinicalTrials.gov 上注册了多项高雄激素血症和多囊卵巢综合征治疗的临床试验。
• The long-term metabolic and cardiovascular outcomes of women with SAHA syndrome are not well characterizedSAHA 综合征女性的长期代谢和心血管结局尚未充分描述。

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