Ashy dermatosis with significant perivascular and subepidermal fibrosis.
Ashy dermatosis with significant perivascular and subepidermal fibrosis.
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灰白色皮肤病，伴有明显的血管周围和表皮下纤维化。
DOI：
10.1097/dad.0b013e31817fb734
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发表时间：
2008
期刊：
影响因子：
--
通讯作者：
C. Monteagudo
中科院分区：
文献类型：
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作者：
J. Martín;V. López;E. Jordá;C. Monteagudo
编者按：持久性色素异常性红斑或灰白色皮肤病是一种病因不明的慢性色素沉着性疾病。它表现为位于躯干和四肢近端的灰色斑点，通常无症状。组织学检查结果显示苔藓样皮炎，基底细胞空泡化，胶质体，真皮乳头内淋巴细胞中度浸润，并混有噬黑素细胞。一位80岁男性来我科就诊，有4个月的无症状病史，在躯干和近端四肢出现蓝灰色椭圆形斑疹，大小为3 - 4 cm（图1）。无光敏性或先前红斑病史，达氏征阴性。皮肤镜检查显示弥漫性分布的小灰色色素斑，在某些区域以精细的线性图案排列，以及类似退化或瘢痕的白色区域（图2）。组织学上，表皮轻度增生，基底细胞色素沉着过度。许多黑素噬菌体存在于浅表血管周围，几乎没有淋巴细胞浸润。此外，还观察到明显的血管周围和表皮下纤维化。Masson-Fontana染色证实了巨噬细胞内存在的色素的黑色素性质（图3）。持久性色素异常性红斑，也称为灰色皮肤病，是一种起源不明的良性疾病，在拉丁美洲人群中更常见。临床上，它的特点是灰色，椭圆形或圆形，无症状的斑疹，可在几个月内消失。这些病变尤其累及躯干和近端肢体，并向外周延伸，通常不累及手掌、足底、头皮、指甲和粘膜。活动性边界的组织病理学结果可能显示基底细胞层空泡变性、偶见胶体、色素失禁、吞噬细胞、充血和扩张的血管以及血管周围炎性淋巴组织细胞浸润。纤维化还没有被报道作为诊断灰色皮肤病的组织学特征。必须排除固定性药疹、色素性扁平苔藓、炎症后色素沉着过度、特发性发疹性黄斑色素沉着和弥漫性色素沉着过度的皮肤病，如阿狄森病或血色病。几种治疗方法，如局部类固醇或全身皮质类固醇，抗生素，紫外线疗法，氨苯砜，或氯法齐明已被尝试与不一致的疗效。这个病例是有趣的，因为皮肤镜检查和病理结果之间的相关性。皮肤镜检查显示的色素呈点状和线状排列，与组织学上观察到的嗜黑症相一致。否则，皮肤镜下观察到的与退化或瘢痕相似的白色区域在组织学上与乳头状真皮和周围血管中存在的显著纤维化相对应。据我们所知，没有以前的报告强调这种相关性，否则可能有助于诊断这种疾病。结论皮肤镜检查可为灰色皮肤病的诊断提供有用的线索。
To the Editor: Erythema dyschromicum perstans or ashy dermatosis is a chronic hyperpigmentary disease of unknown etiology. It presents as ash-colored usually asymptomatic macules located in trunk and proximal extremities. Histopathological findings demonstrate lichenoid dermatitis with vacuolization of basal cells, colloid bodies, and a moderate infiltrate of lymphocytes in the papillary dermis admixed with melanophages. An 80-year-old man presented to our department with a 4-month history of asymptomatic, progressively increasing in number, blue-gray oval macules over his trunk and proximal extremities, which ranged in size from 3 to 4 cm (Fig. 1). No history of photosensitivity or preceding erythema was found. Darier’s sign was negative. Dermoscopy showed small grayish spots of pigment in a diffuse distribution and arranged in a fine linear pattern in some areas, together with whitish areas mimicking regression or scarring (Fig. 2). Histologically, the epidermis was slightly hyperplastic with basal cell hyperpigmentation. Numerous melanophages were present around superficial blood vessels, with virtually absent lymphocytic infiltration. Besides, significant perivascular and subepidermal fibrosis was also noted. Masson–Fontana staining confirmed the melanin nature of the pigment present within macrophages (Fig. 3). Erythema dyschromicum perstans, also referred to as ashy dermatosis, is a benign disease of unknown origin that occurs more frequently in the Latin American population. Clinically, it is characterized by ash-colored, oval or round, asymptomatic macules that may disappear within several months. These lesions especially involve the trunk and proximal extremities and extend peripherally, usually sparing palms, soles, scalp, nails, and mucous membranes. The histopathologic findings from the active border may show a vacuolar degeneration of the basal cell layer, occasional colloidal bodies, pigment incontinence, phagocytic cells, congestive and dilated vessels, and a perivascular inflammatory lymphohistiocytic infiltrate. Fibrosis has not been reported as a histological feature for the diagnosis of ashy dermatosis. It is necessary to exclude fixed drug eruptions, lichen planus pigmentosus, postinflammatory hyperpigmentation, idiopathic eruptive macular pigmentation, and dermatosis that present with diffuse hyperpigmentation such as Addison disease or hemochromatosis. Several treatments such as topical steroids or systemic corticosteroids, antibiotics, ultraviolet light therapy, dapsone, or clofazimine have been tried with an inconsistent efficacy. This case is interesting because of the correlation between the dermoscopic and pathologic findings. The pigment showed by dermoscopy arranged in spots and in a fine linear pattern corresponds to the melanophagia appreciated histologically. Otherwise, the white areas similar to regression or scarring appreciated dermoscopically would correspond histologically with the significant fibrosis present in the papillary dermis and surrounding vessels. To our knowledge, there are no previous reports that emphasize this correlation that otherwise could be helpful for the diagnosis of this disease. As a conclusion, dermoscopy could add helpful clues for the diagnosis of ashy dermatosis.