卡波西样血管内皮细胞瘤1例

报告1例卡波西样血管内皮细胞瘤。患儿男,13个月。右下腹红斑,硬结伴疼痛9个月,皮诊进行性扩大。皮肤科检查见右下腹大片浸润壮斑块。组织病理检查示真皮及皮下组织内长梭形细胞及椭圆形细胞聚集成团块,伴管腔形成,免疫组化示CD31及CD34阳性,诊断为卡波西样血管内皮细胞瘤。     

Kaposiform hemangioendothelioma without Kasabach-Merritt phenomenon

Kasabach-Merritt phenomenon is a serious coagulopathy associated with kaposiform hemangioendothelioma (KHE), tufted angioma, and possibly other vascular neoplasms. KHE presenting in the absence of Kasabach-Merritt phenomenon is rare, although tufted angioma frequently occurs without thrombocytopenia. We retrospectively reviewed 10 cases of KHE without Kasabach-Merritt phenomenon. The tumors appeared as soft tissue masses with the overlying skin being either normal, erythematous, or violaceous. There were no radiologic or microscopic differences in noncoagulopathic KHE as compared with coagulopathic KHE. Evidence of platelet trapping and hemosiderin deposition was seen histologically, despite normal serum platelet levels. All KHE were less than 8 cm in diameter, suggesting that tumors that grow no larger than this size are less likely to trap platelets in sufficient quantity to cause thrombocytopenia. Our series confirms that KHE appears with a wide spectrum of behavior and response to treatment. The decision as to whether or not to treat a noncoagulopathic KHE should be based on the size and location of the tumor and the possible side effects of therapy.     

Transformation between Kaposiform hemangioendothelioma and tufted angioma

Kaposiform hemangioendothelioma and tufted angioma are viewed as two separate disease entities in the current classification of vascular tumors. However, the concurrence of features of both vascular tumors in the same specimen of some patients raises the question whether these tumors exist on a continuum. Herein, we report a dynamic transformation between both tumors within a single patient, which suggests they are two variants of the same vascular tumor.     

Rudimentary polydactyly in an adult: an unusual presentation to a dermatological surgery unit

Accessory digits or polydactyly present uncommonly to dermatologists. Usually they are treated by ligature in the neonatal period or by surgery in childhood. We report a 38-year-old man who presented to a dermatological surgery unit for treatment of a lifelong lesion on the ulnar aspect of his right fifth finger. Clinically this appeared to be a miniature accessory digit and this was discussed with the patient. Subsequent histological examination of the excision specimen confirmed the clinical diagnosis. Further questioning of the patient revealed a strong family history of polydactyly.     

Neutrophilic eccrine hidradenitis with an unusual presentation

Neutrophilic eccrine hidradenitis (NEH) is an eruption most commonly seen in patients undergoing chemotherapy, and is characterized histologically by a neutrophilic infiltrate around the eccrine coils, with associated necrosis of the sweat coils. The rash usually affects the trunk and extremities, and the morphology of the lesions is very variable. We describe a patient with neutrophilic eccrine hidradenitis who presented with symmetrical, erythematous. swollen ears, a manifestation of NEH which has not been previously reported.     

伴Kasabach-Merritt现象卡波西形血管内皮瘤和丛状血管瘤的临床及病理特征

目的 探讨伴Kasabach-Merritt现象(KMP)的卡波西形血管内皮瘤(KHE)和丛状血管瘤(TA)临床病理特征.方法 对7例伴KMP的KHE和TA病例进行临床及病理学分析.结果 诊断为KHE 5例,TA 2例,均伴有血小板降低.7例均在出生后1月内发病,病灶均位于躯干或四肢.2例快速增大,2例缓慢进展,3例稳定或逐渐消退.二者病灶外观多样,典型表现为暗红或紫红色肿块或斑片.组织学上,KHE和TA均由结节状梭形血管内皮细胞团构成.区别在于KHE内皮细胞团边界不清,红细胞淤滞,含铁血黄素沉积;TA内皮细胞团界限清晰,呈弹坑样分布.口服泼尼松治疗2例,切除植皮1例,效果满意;随访观察4例,病情无进展.结论 KMP病理性质为KHE或TA,前者为中间性肿瘤,具局部侵袭性,后者为良性肿瘤,二者均可部分白行退化,需与其他类型血管性肿瘤鉴别.     

伴Kasabach-Merritt现象卡波西形血管内皮瘤和丛状血管瘤的临床及病理特征

Objective To investigate the clinical and pathological features of Kasabach-Merritt phenomenon(KMP)associated with Kaposiform hemangioendothelioma(KHE)and tufted angioma(TA).Methods Clinical presentations and pathological features of seven patients with KHE or TA complicated by KMP were analyzed retrospectively and a literature review was made.Results Five patients were diagnosed with KHE and two with TA,and all of them exhibited a decrease in platelet count(less than 100×109/L).In all patients,lesions were present or developed on the trunk or limbs at birth or wimin one month after birth.The lesions showed a rapid growth in two patients,slow expansion in two patients,kept stable or regressed gradually in three patients.Lesion appearance varied in KHE and TA,and characteristic manifestation was dark-erythematous or prunosus tumors or patches involving the skin or subcutis.Histopathologically.both KHE and TA were composed of numerous fusiform endothellal cell clumps arranged in a lobular fashion.The nodules of KHE had an indistinct boundary usually with erythrocyte stasis or hemosiderin deposition,and TA was characterized by rounded,well-defined nodules distributed in a crater-like fashion within the dermis.A satisfactory outcome was achieved in two patients treated with oral dehydrocortisone and one patient with surgical resection plus skin grafting;the other four patients remained untreated and no progress was observed during follow-up.Conclusions KMP is pathologically associated with KHE or TA.KHE is an intermediate tumor with local aggressiveness while TA is a benign tumor.Both of them can regress spontaneously and should be difierentiated from other types of vascular tumors.     

Oral melanoma: an unusual presentation

Melanoma of the oral cavity is a rare malignant disease that is often discovered in an advanced stage; survival time is an average of five years. Oral melanoma makes up from 0.2 to 8 percent of all melanomas. The presentation of this clinical case demonstrates the complexity of clinical and histopathological manifestations and poor prognosis of oral melanoma.     

Double lip: an unusual presentation

Double lip is a term used to describe a deformity of the upper or lower lip and consists of an accessory fold of redundant mucous membrane inside the vermilion border. Double lip is an uncommon congenital anomaly and is usually diagnosed and treated by a dentist or an oral surgeon. We present an unusual case of double lip--unusual because it was diagnosed and treated by a dermatologist and because of the histopathologic findings.     

Schamberg's disease - an unusual presentation

A 28-year-old man with typical lesions of Schamberg's progressive pigmented purpuric dermatosis involving his right forearm is reported here for its unusual localisation.     

Lipoid proteinosis: an unusual presentation with verruca vulgaris

Lipoid proteinosis (LP) is a very rare, autosomally recessive, inherited disease. It is also known as Urbach Wiethe disease or Hyalinosis cutis et mucosa. LP usually runs a benign, stable, and slowly progressing course. We present one LP case with additional unusual dermatologic involvement; verruca vulgaris was superimposed on papular lesions of LP. To our knowledge, this case is the first and only one described in the literature. In this presentation, we discuss the possibility of predisposition to Human Papilloma Virus (HPV) infections in LP.     

Disseminated rhinosporidiosis - an unusual presentation with pulmonary involvement

BACKGROUND AND AIM: Rhinosporidiosis is a chronic granulomatous disease that commonly affects the nasal and nasopharyngeal mucosa. It rarely presents as disseminated disease. CASE REPORT: We describe a rare case of a patient who had undergone treatment for recurrent nasal rhinosporidiosis and who presented with multiple subcutaneous swellings and pulmonary lesions.