keratin(keratinocyte)快来看

透明细胞棘皮瘤（Degos）是一种少见的，多为单发的肿瘤，好发于中老年人，罕见于年轻人。偶可多发或者播散，皮损常发生在下肢，为境界清楚的粉红色至鲜红色或棕色卵圆形丘疹或结节，直径1-4cm。

 

Clear cell acanthoma透明细胞棘皮瘤Clinical features临床特征Clear cell acanthoma (Degos) is an uncommon, usually solitary

, tumor occurring in the middle aged or elderly but which may rarely present in younger patients. Occasional multiple or disseminated eruptive variants have been described. It is most commonly found on the lower limbs and presents as a circumscribed pink to bright red or brown oval-shaped papule or nodule measuring 1–4 cm in diameter

(Fig. 24.31). Rarely, lesions have been described at other sites including the face, forearm, trunk, inguinal region

, scrotum, buttocks, hallux, and nipple.透明细胞棘皮瘤（Degos）是一种少见的，多为单发的肿瘤，好发于中老年人，罕见于年轻人偶可多发或播散，皮损常发生在下肢，为境界清楚的粉红色至鲜红色或棕色卵圆形丘疹或结节，直径。

1-4cm(图24.31) 。皮损偶可发生在其他部位，如面部、前臂、躯干、腹股沟、阴囊、臀部和乳头。

Fig. 24.31Clear cell acanthoma: typical lesion on the shin of an elderly female. 透明细胞棘皮瘤：在老年女性胫骨的典型皮损。

Occasionally, clear cell acanthoma presents as a polypoid lesion and large variants measuring up to 6 cm have been reported. Rare pigmented variants are also recognized. It often shows a collarette of scale

, and erythematous puncta that bleed with minor trauma are commonly present on the surface.5 Clinically

, it may resemble a pyogenic granuloma or an eccrine poroma. Individual case reports describe clear cell acanthoma arising within an epidermal nevus

, in association with a melanocytic nevus, in a split-thickness skin graft and within a psoriatic plaque. Multiple lesions have been reported in association with ichthyosis.

有时透明细胞棘皮瘤表现为息肉状损害，有报道称大的皮损直径可达6cm也有极个别为深色素性损害皮损常有领口状脱屑及红点，轻微外伤后易出血临床表现与化脓性肉芽肿或小汗腺汗孔瘤类似有个例报告透明细胞棘皮瘤在表皮痣上发生，与黑素细胞痣有关，在中厚植皮以及银屑病斑块上有出现。

多发性损害则与鱼鳞病有关Pathogenesis and histological features发病机制和组织学特征The precise nature of the clear cell acanthoma is unknown. Although variably regarded as an inflammatory epithelial hyperplasia

, a hamartoma or a variant of a seborrheic keratosis, most authors believe it to be a benign neoplasm

, although the cell of origin is subject to dispute. Derivation from epidermal, sebaceous, and sweat gland epithelium have all been proposed. The high glycogen content coupled with keratin and involucrin positivity and carcinoembryonic antigen

(CEA) negativity has led some authors to suggest an origin from the follicular outer root sheath. However

, considering the characteristic follicular sparing this seems an unlikely hypothesis. The presence of striking epithelial membrane antigen

(EMA) expression makes an acrosyringeal derivation most improbable. Recently, the results of lectin binding suggest that it is of epidermal derivation. Furthermore

, immunohistochemical studies on cytokeratin, involucrin, and filaggrin expression support an epidermal derivation and raise the possibility that clear cell acanthoma may represent an inflammatory dermatosis rather than a true neoplasm. This hypothesis

is further supported by recent studies suggesting that, similar to psoriasis, keratinocyte growth factor

(KGF) up-regulation may be responsible for keratinocyte hyperproliferation in clear cell acanthoma.本病的确切性质尚不清楚。

有学者认为，它是一种炎性表皮增生，错构瘤或者脂溢性角化亚型，尽管细胞来源有争论，但绝大多数作者认为是良性肿瘤推测其可能来源于表皮、皮脂腺和汗腺上皮它富含糖原、角蛋白、外皮蛋白呈阳性且癌胚抗原阴性，因此一些作者认为其来源于毛囊外毛根鞘。

但是毛囊的特征性表现缺乏，因此假设难以成立由于显著的上皮膜抗原（EMA）表达，因此末端汗管来源也不大可能近来发现植物凝集素结合，提示可能是表皮来源此外，细胞角蛋白、外皮蛋白和丝聚蛋白的免疫组化研究进一步支持表皮来源说，并且提出透明细胞棘皮瘤可能是一种炎症性皮肤病，而不是真性肿瘤。

最近的研究表明，与银屑病相似，角质形成细胞生长因子（KGF）的上调可能与透明细胞棘皮瘤的角质形成细胞增生有关It is composed of markedly acanthotic (often psoriasiform。

) epithelium, which has a characteristically clearly demarcated lateral border (Fig. 24.32). The epidermal ridges are commonly fused. Individual cells have clear cytoplasm due to the presence of abundant glycogen

, best demonstrated with a periodic acid-Schiff (PAS) reaction (Fig. 24.33). Variably pigmented, dendritic melanocytes are sometimes present

, both along the basal epithelial layer and also intermingled with keratinocytes in the upper layers of the lesion. This latter feature appears to be more common in patients of Mediterranean ancestry.  In cases where pigmentation is clinically apparent

, the confusing term clear cell melanoacanthoma (cf. seborrheic keratosis) or pigmented clear cell acanthoma has sometimes been applied. Typically

, the intraepidermal portions of the adnexae are spared. Intralesional neutrophils are characteristic and are often evident within an overlying parakeratotic scale. The underlying dermal papillae commonly contain dilated capillaries

, and an inflammatory cell infiltrate with a predominance of neutrophils is often present. These latter features show considerable overlap with psoriasis.

透明细胞棘皮瘤有显著的棘层增厚(常呈银屑病样)与两侧上皮分界清楚是其特征(图. 24.32)表皮突通常是融合的由于细胞质内富含丰富糖原（PAS染色可证实），因此细胞透明(图. 24.32)可见数量不等的树突状黑素细胞，沿基底层分布，并混杂在其上表皮中。

后一种情况在地中海血统的患者中更常见对于临床表现为深色素的病例称其为透明细胞色素棘皮瘤（和脂溢性角化相比）或色素性透明细胞棘皮瘤，这是容易产生混淆的术语附属器的上皮部分通常不受累，病灶中有中性粒细胞浸润是其特征，该区域上有角化不全，真皮乳头常有毛细血管扩张，可见以中性粒细胞为主的炎性细胞浸润。

这些改变与银屑病重叠Recently, so-called atypical clear cell acanthoma has been documented in which cellular pleomorphism and mitotic activities are features.34 Whether

, in reality, this represents a true variant is uncertain.近来，有报告所谓非典型性透明细胞棘皮瘤，特征为细胞多形性和分裂活跃然而事实上，这是否代表一种真的变型也不能确定。

图24.32-A

Fig. 24.32-BClear cell acanthoma: (A) the lesion, which is sharply demarcated, shows striking hyperplasia

;(B) although the basal epithelial cells retain their normal tinctorial properties, most of the epithelium shows marked pallor.

透明细胞棘皮瘤：（A）皮损有比较清楚的边界和显著的增生。（B）尽管基底细胞保留其原有的着色性能，但大多数的表皮表现为明显的苍白。

图24.33-A

Fig. 图24.33-BClear cell acanthoma:(A) neutrophils in transit and within the crust on the surface of the lesion are characteristic

;(B)the keratinocyte pallor is due to massive glycogen deposition as seen in this periodic acid-Schiff reaction.

透明细胞棘皮瘤：（A）皮损表面的痂皮中含有或者转运中的中性粒细胞是特征样表现；(B)角质形成细胞苍白的原因是大量的糖原沉积(可见于PAS染色)––––––––––––––––––––––––––––––––––。

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