Rombo Syndrome

Rombo syndrome is characterized by vermiculate atrophoderma, milia, hypotrichosis, trichoepitheliomas, peripheral vasodilation with cyanosis and basal cell carcinomas.

Epidemiology

It has been described in four generations of one family and in two additional sporadic cases.

Clinical description

The skin lesions become visible between 7 and 10 years of age and are most pronounced on the face. Basal cell carcinomas are frequent and develop at around 35 years of age.

Etiology

The etiology remains unknown.