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伴有假性黑素瘤特徵的硬化性痣
臨床特徵 伴有假性黑素瘤特徵的硬化性細胞痣是近期被描述的一種疾病，臨床和組織病理上類似於淺表擴散型黑素瘤。其也被稱為「退行樣纖維增生性色素痣」 (NRLF)。這種類型的色素痣的病因是由於在先前色痣處輕微或忽視的創傷引發的。組織病理學上，NRLF具有三種類型：(i)非典型交界增生，並伴有Paget樣擴散；(ii) 真皮硬化顯著部位可見結構化非典型黑素細胞巢; (iii) 殘餘痣組織（通常伴有先天性特性）環繞並深入到瘢痕組織。根據Fabrizi等人，NRLF可通過缺乏細胞學異型性，細胞分裂相，細胞壞死，腫瘤性黑素沉著和非典型黑色素細胞的膨脹性生長的皮膚結節與擴散型黑色素瘤相區別。其好發於中青年男性，主要累及背部隆起區域，如肩胛區，其可能由於慢性創傷好發於該部位。
皮膚鏡 退行樣纖維增生性色素痣為非典型色素性皮損，表現為表面不同程度（10%-50%表面積）和多色（同時存在白色和藍色）的退行性特徵，但缺乏黑素瘤特徵性表現。有一些特點可以增加NRLF的疑診指數：青年/中年患者；皮損位於背部凸出部位；中心對稱分布的擴散；藍色或者多色的退行變；局部限性退行變（背部凸出區域＜50%:；其他區域＜10%）（圖4）
Figure 4 Sclerosing nevus with regression like fibrosis. (a) Clinical overview of the abdomen of a young man with multiple nevi. (b) Close up of a flat, light brown, 1 cm in diameter lesion. (c) Dermoscopy, displaying an atypical pigmented lesion, with features of regression which is extensive and polychromatic (coexisting white and blue areas). Inverse network is visible at the periphery of the lesion.
反射式共聚焦顯微鏡 迄今為止尚無介紹退行樣纖維增生性色素痣的文獻。本例病人RCM顯示存在邊界不清的皮損，伴有真皮表皮交界處增厚以及眾多的噬黑素細胞。在RCM中，細胞異型和局部Paget樣擴散並不能與退行性黑色素瘤進行準確的鑑別診斷，在組織病理學中也經常遇到這些問題。（圖5）
Figure 5 Reflectance confocal microscopy imaging of a sclerosing nevus. (a) Mosaic image at the level of the DEJ shows a hill circumscribed lesion, with junctional thickening, visible as elongate, enlarged, junctional nests (white arrows). (b) Close up of melanocytic nests, intermingled with roundish to oval plump bright cells corresponding to melanophages (red arrows) (c) Focal area of partial disruption of the rete ridges, with collagen bundles and numerous melanophages.
圖5 硬化性痣在反射式共聚焦顯微鏡的圖像。(a)真皮表皮交界處的拼接圖像顯示，交界處增厚，可見延長，擴大的交接巢（白色箭頭）。（b）黑素細胞巢，夾雜有圓形至卵形豐滿明亮的細胞（對應於噬黑素細胞）（紅色箭頭）。（c）表皮突部分損壞的聚焦部位，伴有膠原束和眾多的噬黑素細胞。
管理 由於NRLF是黑素瘤模仿者，因此常規切除這些皮損以排除黑素瘤。
靶樣痣臨床特徵 該痣主要特徵為中央粉紅至深色色素沉著，通常為丘疹，其與周圍較深的色素沉著邊緣之間被環狀的正常皮膚分隔。僅有少數文獻對徽章痣進行了報導，因此，其被認為是罕見；然而，就作者經驗而言，與文獻所提及的數量相比，該病在生活中更為普遍。大多數病例報導其出現在兒童和青少年的頭皮上，引起臨床醫生的長期關注。在組織學上，中央痣為交界痣或複合痣，介於中間的非色素性區域不含痣細胞，外圍暈是由交界巢組成。
皮膚鏡 根據文獻資料，僅有少數病例報告介紹了徽章痣的皮膚鏡特徵。所有患者均為青少年，顯示中央為球狀或均質圖像，較淺的均質內環，以及深色網狀外環。
Figure 6 Cockade nevus (a, b) and Halo nevus (c, d). (a) Clinical image of a large pigmented lesion on the back of an adolescent patient. (b) Dermoscopy revealing a darker, central homogeneous pattern, a lighter homogenous inner ring, and a peripheral darker reticular ring. (c) Clinical image of a pigmented lesion on the back of a young girl, surrounded by a white peripheral halo. (d) Dermoscopically, the nevus exhibit a globular pattern, which is surrounded by a rim of a white regression-like depigmentation.
圖6 徽章痣（a, b）和暈痣（c, d）。（a）1例青少年患者背部大的色素性皮損臨床圖片。（b）皮膚鏡顯示中央為深色均質圖像，較淺的同質內環和深色網狀外環。（c）1名年輕女性背部色素性皮損的臨床圖片，皮損周圍繞以白色暈。（d）從皮膚鏡上來說，暈痣顯示為球狀圖像，周圍繞以白色退行性脫色暈。
反射式共聚焦顯微鏡 尚無報導帽章痣反射式共聚焦顯微鏡特徵的文獻。
處理 由於徽章痣為良性，因而無需進一步治療。
Sutton痣（暈痣）
臨床特徵 暈痣（HN）也稱為Sutton痣或離心性後天性白班，是一種良性黑素細胞痣，周圍繞以環狀脫色區，似暈樣。HN常見於兒童和青年，發作的平均年齡為15歲，無性別或種族偏好。估計HN在人群中的發病率約為1%。受累者經常出現多個皮損，且通常位於背部。25–50%的暈痣患者可發展為多發性暈痣。多為複合痣，儘管交界痣或真皮痣也有可能。先天性痣和獲得性痣均可受累。最常提及的暈痣觸發因素為壓力（60%）和青春期（40%）。此外，已報導了HN的家族遺傳性，其可能與特應性皮炎或自身免疫性疾病（如白癜風和橋本甲狀腺炎）有關。暈痣是白癜風的標誌，還是白癜風的一個危險因素？這一問題仍存在爭議。在它們的發展過程中，可能遭受從原始的黑素細胞痣到部分和全部退化（最終僅存暈圈）的漸進性臨床階段。據報導，至少50%的患者的中央痣最終會完全消失。HN通常持續10年或更久。某些可在退化階段回歸至正常皮膚，但即便如此，這些皮損仍會平均持續7.8年。
組織病理學發現真皮內有大量淋巴細胞苔蘚樣浸潤，伴有痣細胞巢或位於炎性細胞中的單個痣細胞。白色暈處顯示基底層黑色素和黑素細胞缺失。
皮膚鏡 中央痣組成部分通常為球狀和/或均質圖像，周圍繞以不同的環狀白色退行性脫色區。少見中央痣為網狀圖像。正如前文提到的臨床階段，中央痣組成部分可能完全缺乏，在這種情況下，可能存在中央紅色色素沉著，為最終來自真皮血管叢。隨訪期間，HN可能出現相當大的變化。一項研究依序對暈痣進行電子皮膚鏡檢查，顯示51.5%的暈圈尺寸減小，然而27.3%的暈圈尺寸增大。即便痣變小，其皮膚鏡特徵仍保持不變（圖6）。
反射式共聚焦顯微鏡 近期，僅一項病例系列報告介紹了暈痣的反射式共聚焦顯微鏡特徵。作者介紹了9例臨床診斷為HN的患者，在5例（55.6%）患者中，可觀察到靶樣細胞，在3例（33%）患者中，可發現無邊緣的真皮乳頭和表皮真皮交界處增厚。在7例（77.8%）和6例（66.7%）患者中分別發現真皮乳頭層的有核細胞以及豐滿明亮的細胞。作者推斷，HN的RCM檢查可顯示出非典型特徵，且與在非典型黑素細胞皮損和惡性黑素瘤中所觀察到的特徵重疊。
Figure 7 Reflectance confocal microscopy (RCM) of a halo nevus. (a) RCM mosaic image at the level of spinous layer, displaying a very well well-circumscribed lesion. To note, the presence of dense and bright nests at the periphery of the lesion (red arrow), corresponding to peripheral globules in dermoscopy. (b) Corresponding dermoscopy image. (c) Close up of melanocytic nests that appear 『stretched』 (white circle) and intermingled with inflammatory cells. (white arrows).
圖7 暈痣的反射式共聚焦顯微鏡（RCM）圖像。（a）棘層的RCM拼接圖像顯示邊界十分清楚的皮損。需注意皮損周圍存在緻密且明亮的細胞巢（紅色箭頭），其相當於皮膚鏡檢中的外圍小球。（b）相對應的皮膚鏡圖像。（c）黑素細胞巢特寫，可見其出現「拉伸」（白色圓圈）且與炎性細胞混合（白色箭頭）。
處理 在電子皮膚鏡隨訪期間，隨著時間的推移，暈痣面積可能有相當大的變化，儘管其結構圖像仍保持不變。基於這一原因，加上伴有暈狀色素減退的黑素瘤（儘管罕見）表現出黑素瘤特異性的皮膚鏡特徵，因此電子皮膚鏡隨訪在HN診斷中的作用被認為無關緊要。宣教HN的長期自然病程，可使患者安心且避免不必要的切除。
Meyerson痣（溼疹樣痣）
臨床特徵 Meyerson現象是以1個或多個色素痣周圍出現環狀溼疹樣改變為特徵。臨床特徵表現為紅斑性暈，上覆鱗屑，有時紅斑區域的外圍加重。此過程可能僅局限於個體的一個、部分或所有的痣。該病年輕的健康成人多見。大多數皮損出現輕微瘙癢。外用糖皮質激素治療後，溼疹樣皮損脫屑，自發清除或消退。一旦周圍的溼疹消退，痣保持不變。Meyerson推測這種發作是黑素細胞痣周圍的局限性玫瑰糠疹，但考慮到玫瑰糠疹十分常見且Meyreson痣較少見，因此這種關聯並不明顯。多發性Meyreson痣需與玫瑰糠疹和梅毒的玫瑰疹鑑別診斷。
在非黑素細胞性皮損中也出現了這種現象，如基底細胞癌、鱗狀細胞癌、脂溢性角化病、瘢痕疙瘩、灰泥角化病、組織細胞纖維瘤和昆蟲叮咬。組織病理學顯示痣（通常為複合痣）周圍角化不全、棘層肥厚，有時表皮海綿形成。真皮上部有血管周淋巴細胞浸潤；有時可伴有嗜酸性粒細胞。
皮膚鏡 Meyerson現象與相關痣的皮膚鏡特徵一致。然而，淺表黃色的血清痂皮使痣的圖像變得模糊，因此難以對其評估（圖8）。
Figure 8 Meyerson’s nevus. (a) Clinical image of a nevus with peripheral eczematous halo on the upper arm of a 30- year-old lady. (b) Dermoscopy showing yellowish superficial sero-crusts that do not allow a clear imaging on the underling nevus. (c) RCM mosaic showing multiple spongiotic vesicles, seen as round to ovoidal dark spaces, visible in the superficial layers of the epidermis. (white arrows) (d) Close up of one vesicle, fulfilled by small bright roundish particles, corresponding to inflammatory cells.
圖8 Meyerson痣。（a）1例30歲女性上臂伴有溼疹樣暈的痣的臨床圖片。（b）皮膚鏡顯示淺表黃色血清痂皮導致下方痣的圖像不清晰。（c）RCM拼接圖像顯示錶皮淺層可見多個海綿水腫水皰，見圓形至卵形暗區（白色箭頭）。（d）一個水皰的特寫，填充有小的明亮圓形顆粒，相當於炎性細胞。
反射式共聚焦顯微鏡 Meyerson痣的反射式共聚焦顯微鏡圖像可使體內海綿水腫囊泡可視化，典型黑素細胞痣周圍的表皮淺層內可見圓形至卵形暗區（圖8）。
處理 由於局部治療後，炎性改變通常消失，因此，需在數周后重新進行評估以保證其為良性狀態。例外的是，外用糖皮質激素乳膏治療後，一些溼疹可能無法治癒，並且曾有報告提及只有在切除痣後，溼疹才會消退。Sclerosing nevi with pseudomelanomatous features
Clinical features Sclerosing nevus with pseudomelanomatous features is a recently described entity which can clinically and ahistopathologically simulate regressing melanoma. They are also called 『nevi with regression-like fibrosis』 (NRLF). The aetiology of this type of nevus is attributed to a minor or unnoticed trauma(s) on a pre-existing nevus. Histopathologically NRLF exhibit a trizonal pattern: (i) an atypical junctional proliferationassociated with some pagetoid spreading; (ii) significant area(s) of dermal sclerosis containing architecturally atypical melanocytic nests; (iii) residual nevus tissue (often with congenital-like features) around and deep into the cicatricial tissue. According to Fabrizi et al. NRLF can be differentiated from regressing melanoma by lacking cytologic atypia, dermal mitoses, cellnecrosis, tumoural melanosis and expansile dermal nodule(s) of atypical melanocytes. It appears in young to middle-aged men, mostly located on convex area of the back, i.e. the scapular area, probably due to the chronic trauma(s) that frequently occur in this body area.
Dermoscopy Nevi with regression-like fibrosis are pigmented atypical lesion showing overall features of regression, which is invariably extensive (between 10% and 50%) and polychromatic (coexisting white and blue areas), in absence of melanoma specific criteria. There are some criteria that rise the index of suspicion of NRLF: young/middle-aged patients; lesions located in the convex area of the back; symmetric-central distribution of regression; blue or polychromatic regression; limited regression (<50% in the convex area of the back; <10% elsewhere) (Fig. 4).
Reflectance confocal microscopy Nevi with regression-like fibrosis have not been described in the literature up to now. In the present case, RCM showed the presence of a ill-defined lesion, with junctional thickening and numerous melanophages at the dermoepidermal junction (DEJ). In RCM, the presence of cellular atypia and focal pagetoid spread did not allow an accurate differential diagnosis with regressive melanoma, reflecting the difficulties often encountered also in histopathology (Fig. 5).
Management As NRLF is a melanoma simulator，these lesions are usually routinely excised to rule out melanoma.
Targetoid nevi
Cockade nevi
Clinical features This nevus is characterized by a central pink to darkly pigmented, often papular portion, which is surrounded by an inner de-pigmented and outer, pigmented rim. There are only few reports in the literature reporting on cockade nevus and accordingly, this nevus is considered rare, however, in our experience it occurs more commonly than suggested in the literature. In the majority of cases this nevus is reported on the scalp of children and adolescents, where for long time it represented a matter of concern for clinicians. Histologically, the central nevus is of junctional or compound type, the intervening non-pigmented zone is devoid ofnevus cells and the peripheral halo is composed of junctionalnests.
Dermoscopy Dermoscopic pattern of cockade nevus has beendescribed only in a few cases according to the literature.Allcases occurred in adolescents, revealing a darker, central globularor homogeneous pattern, lighter homogenous inner ring and a peripheral darker reticular ring (Fig. 6).
Reflectance confocal microscopy Reflectance confocal microscopy features of cockade nevi have not been reported in the literature.
Management As cockade nevi are benign, no further treatmentis warranted.
Sutton nevus (halo nevus)
Clinical features Halo nevus (HN), also termed Sutton’s nevus or leukoderma acquisitum centrifugum, is a benign melanocytic nevus surrounded by an achromic rim that simulates a halo. HN usually appear in children and young adults, with an averageage of onset of 15 years.There is no predilection for sex orrace.The estimated incidence of HN in the population isaround 1%.Affected individuals frequently have multiplelesions, which are usually localized on the back. Multiple halonevi was developed by 25–50% of halo nevi patients. It involves most commonly compound nevi, although a junctionalor dermal pattern is also possible. Both congenital and acquirednevi can be affected.The most frequently mentioned triggerfactors for halo nevi are stress (60%) and puberty (40%).A familial tendency for HN have been reported as well.Theymay be associated with atopic dermatitis or with autoimmunedisorders such as vitiligo and Hashimoto thyroiditis.The questionwhether halo nevi should be considered as a sign of vitiligo, or as arisk factor for developing vitiligo is still under debate.In their evolution they can undergo progressiveclinical stages from the originalmelanocytic nevi to a partial and total regression that leads to only presence of the halo. It is described that at least 50% of patientseventually have total disappearance of the central nevus.HNtypically persists for a decade or longer. A subgroup may progressthrough stages of involution with a return to normal-appearing skin,but even these lesions persist for an average of 7.8 years.
Histopathologically, a heavy, lichenoid lymphocytic infiltrate within the dermis is noticed, with nevus cells arranged in nests or singly among the inflammatory cells. The whitish halo shows an absence of melanin and melanocytes in the basal layer.
Dermoscopy The central nevus component typically shows a globular and/or homogeneous pattern, which is surrounded by a variable rim of a white regression-like depigmentation. Less oftenly the central nevus component may display a reticular pattern. As mentioned regarding the clinical stage, the central nevus component may lack entirely, and in these cases a reddish central pigmentation eventually revealing visible vessels from the dermal vascular plexus may be present. HN may reveal considerable changes during follow-up. In one study, halo nevi were sequentially followed with digital dermoscopy and 51.5% showed a decrease in halo size, whereas 27.3% exhibited an enlargement in halo size. Dermoscopic pattern of the nevus remained unchanged as it became smaller (Fig. 6).
Reflectance confocal microscopy Reflectance confocalmicroscopy features of halo nevi have been recently describedonly in one case series.Authors describe nine cases with a clinical diagnosis of HN. In five (55.6%) cases, pagetoid cells were observed. Non-edged dermal papilla and junctional thickening were found in three (33%) cases. Nucleated cells in the dermal papillae and plump bright cells were observed in seven (77.8%) and six (66.7%) cases, respectively. Authors conclude that RCM examination in HN can show atypical features that overlap with those observed on atypical melanocytic lesions and malignant melanoma (Fig. 7).
Management Halo nevus reveals considerable changes of area over time during digital dermoscopic follow-up, albeit their structural patterns remain unchanged. For this reason and because melanoma with halo like depigmentation, despite being rare, additionally exhibits melanoma-specific dermoscopic criteria, the role of digital dermoscopic follow-up in the diagnosis of HN was described as insignificant. Education about the prolonged natural history of HN may reassure patients and avoid unnecessary excision.
Meyerson’s nevus (eczematos nevus)
Clinical features Meyerson’s phenomenon is characterized by the development of an eczematous halo around one or more pigmented nevi.Clinical features consist on the appearance of erythematous halos with overlying scales sometimes accentuated at the peripheries of the erythematous zones. This process can be confined to one, some or all nevi of an individual. It is more frequent in young healthy adults. Slight pruritus is a feature in most lesions. Eczematous lesions become desquamative and clear spontaneously or resolve under topical therapy with corticosteroids. The nevus persists unchanged once the surrounding eczema had resolved. Meyerson postulated this eruption to be the localization of pityriasis rosea around melanocytic nevi, but this association is not clear considering that pityriasis rosea is very frequent and Meyreson’s nevi are relatively rare. In multiple Meyerson’s nevi, the differential diagnosis are pityriasis rosea and the roseola of syphilis.
This phenomenon has been also described in non-melanocytic lesions such as basal cells carcinomas, squamous cell carcinomas, seborrheic keratosis, keloids, stuccokeratosis, histiocytofibromas and insect bites. Histopathology shows a nevus, generally compound, with associated parakeratosis, acanthosis and sometimes epidermal spongiosis. Upper dermis has a perivascular lymphocytic infiltrate; sometimes with eosinophils.
Dermoscopy Meyerson’s phenomenon does not modify dermoscopic characteristics of involved nevi. Nevertheless,patterns are often blurred by a yellowish overlying superficial serocrust and therefore are difficult to assess (Fig. 8).
Reflectance confocal microscopy Reflectance confocalmicroscopy imaging of Meyerson’s nevi allows the visualization of spongiotic vesicles in vivo, as round to ovoidal dark spaces, visible in the superficial layers of the epidermis around a typical melanocytic nevus (Fig. 8).
Management As the inflammatory changes almost always clear with topical treatment, re-evaluation after some weeks should be performed to reassure the presence of benign nevus pattern. Exceptionally eczema may not be cured after treatment with topic corticosteroids creams and clarifying of the eczema only after the excision of the nevus was described.
由MediCool醫庫軟體塗秀麗 編譯，上海市皮膚病醫院陳裕充博士審核
原文來自：JEADV 2014, 28, 833–845