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The prevalence is unknown but around 200 cases have been described in the literature. However, not all cases of tufted angioma are published and a certain number of cases are probably misdiagnosed as noninvoluting congential haemangiomas (NICH).

Tufted angiomas appear as poorly delineated red plaques, sometimes containing scattered deep-red papules. In rare (usually congenital) cases, tumoural masses have been reported. The appearance of the lesions changes with time and they are sensitive upon palpitation, or even painful if subjected to trauma. The angiomas are sometimes covered by a layer of downy hair or encircled by a clear ring of tissue or halo.

The diagnosis relies on the histological findings, which are characterised by a cannonball distribution of tufts of capillaries distributed throughout the dermis in a lobular arrangement. Lymphatic spaces and dermal fibrosis are frequently associated. Tufted angiomas are GLUT1-negative tumours that can be clearly distinguished from infantile haemangiomas.

General corticotherapy, or therapy with alpha interferon or vincristine may be beneficial in severe cases with an extendedclinical course.

Although rare cases of spontaneous regression have been reported, the clinical course is often chronic. In some cases, tumours arising in the lower limbs infiltrate and progressively form a hardened (sclerous) tegument. This sclerosis may eventually reach the underlying muscle and joints, leading to significant functional problems. Tufted angiomas may be associated with Kasabach-Merritt syndrome (KMS), in which thrombopaenia can be life-threatening.