Effectiveness of topical adapalene in Dowling-Degos disease
Effectiveness of topical adapalene in Dowling-Degos disease
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DOI：
10.1159/000018102
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发表时间：
1999-01-01
期刊：
影响因子：
3.4
通讯作者：
Frigerio, E
中科院分区：
文献类型：
--
作者：
Altomare, G;Capella, GL;Frigerio, E
Dowling-Degos病(DDD)的临床方面在“屈曲网状色素异常”的描述性名称中有明确的概括。DDD是一种无害但美观的、缓慢演变的巨大皮肤皱褶的色素沉着，具有网状毛囊图案。这种滤泡模式可能非常明显，因此在某些情况下，首字母缩写DDD应该更恰当地意味着‘暗点病’[1]；这个最后一个术语指的是典型的粉刺样黑色针头大小的过度角化毛囊病变[1]。这种疾病主要影响女性，被认为是一种常染色体显性遗传的基因决定的疾病[2]，尽管许多病例似乎是零星的[3]，可能是因为低外显率或不完全的家系调查。现有文献记载了这种综合征与其他罕见的色素异常的关系。然而，到目前为止，治疗方面似乎被忽视了。我们报告一名妇女与公开瘙痒形式的DDD成功地应用阿达帕林治疗。据我们所知，这是第一个令人满意的DDD药物治疗尝试的报告。一位76岁的妇女因乳房下、臀间肌和腹股沟屈曲的丘疹毛囊过度色素沉着入院(图2)。1)。色素斑块内点缀着粉刺样病变。皮疹已经持续了一年，而且非常痒。无掌侧或面部凹陷，无表皮脊断裂，无大汗腺炎。患者的亲属都没有受到影响。这种疾病以前被标记为屈曲性Darier病、黑棘皮病和炎症后色素沉着。以前的治疗方法包括外用类固醇、抗生素和抗真菌药物，以及口服依曲替酸酯(25 mg Bid，3个月)，所有这些都被证明无效。实验室检查，包括内分泌功能测试，都是正常的。患者既不肥胖，也不高血压。行活组织检查。组织学检查显示局灶性角化后阻塞滤泡漏斗，不规则棘突，网脊延长，基底色素沉着，黑素细胞数量正常。血管周围可见稀疏的混合圆形细胞浸润。临床诊断为DDD。开始使用0.1%的阿达帕林凝胶进行局部治疗，仅治疗5天后，患者的情况如图2所示。进一步的改善，包括抑制瘙痒和进一步清除图2中仍然明显的黑暗和更深的网状结构，来自阿达帕林的持续应用，但不能因为迅速复发而停止使用。
The clinical aspect of Dowling-Degos disease (DDD) is clearly summarized in the descriptive denomination ‘reticulate pigmented anomaly of the flexures’. DDD is a harmless but esthetically disfiguring, slowly evolving pigmentation of the great skin folds with a reticulate follicular pattern. This follicular pattern can be very pronounced so that in some cases the acronym DDD should more appropriately mean ‘dark dot disease’[1]; this last term refers to the presence of typical comedo-like, black pinhead-sized hyperkeratotic follicular lesions [1]. The condition affects mainly women, and it is believed to be a genetically determined disease with an autosomal dominant inheritance [2], although many cases appear to be sporadic [3], probably because of low penetrance or incomplete pedigree inquiry. The available literature documents the relation of this syndrome with other rare pigmentary abnormalities. However, the therapeutic aspect seems to have been so far disregarded. We report a woman with an overt pruritic form of DDD successfully treated with the application of adapalene. To the best of our knowledge, this is the first report of a satisfying medical therapeutic attempt in DDD.A 76-year-old woman was admitted with a papular follicular hyperpigmentation of submammary, intergluteal and groin flexures (fig. 1). The pigmentary plaques were studded with comedo-like lesions. The eruption had been lasting for 1 year and was intensely itchy. Palmar or facial pits, breaks in epidermal ridge pattern and adenitis of apocrine glands were absent. None of the patient’s relatives was affected. The disorder had previously been labeled flexural Darier’s disease, acanthosis nigricans and postinflammatory hyperpigmentation. Former treatments included topical steroids, antibiotics and antimycotics, and oral etretinate (25 mg bid for 3 months), all of which had proven ineffective. Laboratory investigations, including tests for endocrine functions, were normal. The patient was neither obese nor hypertensive. A biopsy was performed. The histological examination showed focal parakeratotic plugs obstructing follicular infundibula, irregular acanthosis, elongation of rete ridges and basal hyperpigmentation with a normal number of melanocytes. A sparse perivascular mixed round cell infiltrate was present. The clinical diagnosis of DDD was confirmed. Topical treatment with adapalene gel 0.1% bid was started, and after only 5 days of treatment the patient’s condition was as shown in figure 2. Further improvement, including suppression of itching and further clearing of the dark and deeper reticulum still evident in figure 2, has ensued from the continuous application of adapalene, which however cannot be discontinued because of prompt relapse.