Catteau B, Enjolras O, Delaporte E, Friedel J, Brevière G, Wassef M, Lecomte-Houcke M, Piette F, Bergoend H
Service de Dermatologie A, Hôpital Claude Huriez, Lille.
Ann Dermatol Venereol. 1998 Oct;125(10):682-7.
Tufted angioma belongs to the family of vascular skin proliferations. Clustered capillaries is the characteristic histological feature. Classically, tufted angioma is an acquired disease observed in children or young adults with lesions predominating on the neck, the shoulders and the upper trunk.
Four children were seen for flat skin lesions which were either initially or secondarily erythematous. The lesions were observed at birth or within the first weeks of life. In all 4 cases, the lesions progressed by infiltration, leading to sclerous plaques within a few weeks. The hip area was involved in case n(o) 2 and the thigh in the other cases. Pathology reported tufted angioma. A repeated biopsy 4 years after the first biopsy demonstrated the progressive nature of the fibrosis in case n(o) 2. Magnetic resonance imaging, performed in 3 of the 4 cases, demonstrated deep penetration of the vascular proliferation. The lesions were in contact with bony structures in case n(o) 2, reached the hypodermis in case n(o) 3 and infiltrated between the adducter muscles in case n(o) 4.
Among tufted angiomas, there is a clinical variant characterized by very early discovery (congenital or within the first months of life), preferential localization on the lower limbs and progression to sclerosis. Although non-specific, hypersudation and lanuginous hypertrichosis are also highly suggestive signs. The main clinical and histological differential diagnosis is angio-eccrine hamartoma and, to a lesser degree, Kaposi hemangioendothelioma which does not predominate on the lower limbs. This particular clinical presentation of tufted angioma should lead to wide and sufficiently deep biopsy to allow precise diagnosis prior to initiating therapy. The possibility of spontaneous involution would allow abstention with regular surveillance to detect reactivation and progression or functional impairment. Such unfavorable progression has lead certain authors to propose high-dose systemic corticosteroid therapy or alpha-interferon.
簇状血管瘤属于皮肤血管增生性疾病。成簇的毛细血管是其特征性组织学表现。典型的簇状血管瘤是一种后天性疾病，多见于儿童或青年，病变主要位于颈部、肩部和上胸部。
4例儿童因皮肤扁平病变就诊，这些病变起初或继发时为红斑。病变在出生时或出生后几周内出现。在所有4例中，病变通过浸润进展，在几周内形成硬结斑块。2号病例病变累及臀部，其他病例累及大腿。病理报告为簇状血管瘤。首次活检4年后再次活检显示2号病例纤维化呈进行性发展。4例中的3例进行了磁共振成像检查，显示血管增生深入。2号病例病变与骨质结构接触，3号病例病变到达皮下组织，4号病例病变浸润于内收肌之间。
在簇状血管瘤中，有一种临床变异型，其特点是发现非常早（先天性或出生后数月内），下肢为好发部位，且会进展为硬化。多汗和胎毛增多虽然不具特异性，但也是高度提示性的体征。主要的临床和组织学鉴别诊断是血管小汗腺错构瘤，在较小程度上还有卡波西血管内皮瘤，后者在下肢并不多见。簇状血管瘤的这种特殊临床表现应进行广泛且足够深的活检，以便在开始治疗前做出准确诊断。自发消退的可能性使得可以在定期监测以发现复发、进展或功能损害的情况下暂不治疗。这种不良进展促使一些作者提出采用大剂量全身皮质类固醇治疗或α干扰素治疗。
