Daadaa Najla, Sathe Nishad C., Ben Tanfous Azima
Military Hospital of Tunis
Habib Thameur Hospital
Riehl melanosis is an acquired pigmentary disorder that predominantly affects darker-skinned individuals, particularly older women. This condition is characterized by brown-gray reticulated-to-diffuse hyperpigmented macules or patches on the face, neck, and upper chest (see Clinical Photographs of Riehl Melanosis). The etiology is presumed to be a type IV hypersensitivity reaction triggered by contact allergens such as personal fragrances, textiles, and cosmetics. However, genetic factors, autoimmune conditions, and UV light exposure may also contribute to the condition's development. Despite the distinctive clinical presentation of Riehl melanosis, a thorough evaluation is necessary for its differential diagnosis, which may involve various techniques such as skin biopsy, patch testing, dermoscopy, reflectance confocal microscopy, or newer diagnostic tools following an initial clinical examination of the patients. These tools are crucial for diagnosing and distinguishing Riehl melanosis from other differential diagnoses, including conditions such as lichen planus pigmentosus and erythema dyschromicum perstans, all falling under the spectrum of acquired dermal macular hyperpigmentation. Histopathological examination of a skin biopsy will reveal basal layer vacuolar degeneration accompanied by colloid bodies and pigment incontinence. Apart from allergen avoidance, management of this condition involves sun protection and various tailored regular skincare measures. Treatment options include topical therapies (such as hydroquinone, azelaic acid, and retinoids), oral therapies (such as tranexamic acid, glycyrrhizin, and mycophenolate mofetil), chemical peels (such as salicylic acid and glycolic acid), and lasers and light therapy (such as 1064-nm quality-switched neodymium-doped yttrium aluminum garnet laser and intense pulsed light) to meet individual patient needs. Regular monitoring using the dermal pigmentation area and severity index (DPASI) is necessary to gauge treatment efficacy and mitigate potential psychosocial impacts. This involves ensuring regular observation for improvement of hyperpigmentation, which can have deleterious effects if left untreated, particularly in patients with Riehl melanosis. Riehl melanosis was first described by Gustav Riehl in 1917, who observed striking rough, indurated brown-gray pigmentation on the forehead, temporal, and zygomatic regions on patients' faces (see Riehl Melanosis on the Left Cheek). Initially, it was believed to be related to nutritional substitutions resulting from wartime food measures, such as poor-quality flour. However, it later became evident that it was also present on the faces of women who used cosmetic products. Consequently, the condition transitioned from being termed war dermatosis to melanosis faciei feminae, eventually becoming known simply as Riehl melanosis. Riehl melanosis belongs to a group of conditions collectively referred to as acquired dermal macular hyperpigmentation (previously known as macular pigmentation of uncertain etiology). This group may encompass Riehl melanosis, erythema dyschromicum perstans, lichen planus pigmentosus, melasma, exogenous ochronosis, idiopathic macular eruptive pigmentation, and pigmented contact dermatitis. The acquired dermal macular pigmentation disorders have similar clinical and histopathological findings. Pigmented contact dermatitis is considered a variant of Riehl melanosis, with an identified contact allergen, such as cosmetics like kumkum (see Kumkum-Induced Pigmented Contact Dermatitis). Although pigmented contact dermatitis shares similarities with Riehl melanosis, the extent of inflammation observed creates distinctions between them. Some experts consider pigmented contact dermatitis and Riehl melanosis identical; however, this view may be controversial, as pigmented contact dermatitis typically exhibits minimal to no inflammation compared to Riehl melanosis. Thus, this group of conditions may be classified as acquired dermal macular pigmentation with or without contact sensitization. Please see StatPearls' companion resource, "Contact Dermatitis," for further information.
里尔黑变病是一种后天性色素沉着紊乱疾病,主要影响肤色较深的个体,尤其是老年女性。该病的特征是面部、颈部和上胸部出现棕灰色网状至弥漫性色素沉着斑或斑片(见里尔黑变病临床照片)。病因推测是由个人香水、纺织品和化妆品等接触性过敏原引发的IV型超敏反应。然而,遗传因素、自身免疫性疾病和紫外线照射也可能促使该病的发展。尽管里尔黑变病有独特的临床表现,但进行全面评估以进行鉴别诊断是必要的,这可能涉及多种技术,如皮肤活检、斑贴试验、皮肤镜检查、反射式共聚焦显微镜检查,或在对患者进行初步临床检查后使用更新的诊断工具。这些工具对于诊断里尔黑变病并将其与其他鉴别诊断区分开来至关重要,包括色素性扁平苔藓和持久性色素异常性红斑等疾病,它们都属于后天性真皮性斑状色素沉着的范畴。皮肤活检的组织病理学检查将显示基底层空泡变性,并伴有胶样小体及色素失禁。除了避免接触过敏原外,该病的管理还包括防晒和各种定制的常规皮肤护理措施。治疗选择包括局部治疗(如氢醌、壬二酸和维甲酸)、口服治疗(如氨甲环酸、甘草酸和霉酚酸酯)、化学剥脱(如水杨酸和乙醇酸)以及激光和光疗(如1064纳米调Q掺钕钇铝石榴石激光和强脉冲光),以满足个体患者的需求。使用皮肤色素沉着面积和严重程度指数(DPASI)进行定期监测对于评估治疗效果和减轻潜在的心理社会影响是必要的。这包括确保定期观察色素沉着的改善情况,如果不治疗,色素沉着可能会产生有害影响,尤其是里尔黑变病患者。里尔黑变病于1917年由古斯塔夫·里尔首次描述;他观察到患者面部的前额、颞部和颧部区域有明显粗糙、硬结的棕灰色色素沉着(见左脸颊的里尔黑变病)。最初,人们认为这与战时食品措施导致的营养替代有关,比如劣质面粉。然而,后来发现使用化妆品的女性面部也会出现这种情况。因此,该病从被称为战争性皮炎转变为女性面部黑变病,最终简称为里尔黑变病。里尔黑变病属于一组统称为后天性真皮性斑状色素沉着(以前称为病因不明的斑状色素沉着)的疾病。这组疾病可能包括里尔黑变病、持久性色素异常性红斑、色素性扁平苔藓、黄褐斑、外源性褐黄病、特发性斑疹性色素沉着和色素性接触性皮炎。后天性真皮性斑状色素沉着疾病有相似的临床和组织病理学表现。色素性接触性皮炎被认为是里尔黑变病的一种变体,有确定的接触性过敏原,如朱砂等化妆品(见朱砂引起的色素性接触性皮炎)。尽管色素性接触性皮炎与里尔黑变病有相似之处,但观察到的炎症程度使它们有所区别。一些专家认为色素性接触性皮炎和里尔黑变病相同;然而,这种观点可能存在争议,因为与里尔黑变病相比,色素性接触性皮炎通常表现出极少的炎症或无炎症。因此,这组疾病可分为伴有或不伴有接触致敏的后天性真皮性斑状色素沉着。有关更多信息,请参阅StatPearls的配套资源《接触性皮炎》。