Acquired Port-Wine Stain (Fegeler Syndrome): A Report of 3 Cases.

Capillary malformation is a condition included in the group of vascular malformations. It is one of the most common vascular abnormalities and can affect up to 0.3% of newborns. It presents clinically as a homogeneous erythematous macule with well-defined borders. It is typically unilateral and is usually present at birth. However, cases of acquired capillary malformation have been reported in patients with no previous lesions of this type. Patient 1 was a 9-year-old girl followed-up from her first year of life for mastocytosis presenting as urticaria pigmentosa and treated with sodium cromoglycate and cetirizine. When she was 7 years old, a well-defined pink macule started to become visible, affecting segmentally the area of the first branch of the trigeminal nerve, and the superior part of the area of the second branch, with a more violaceous appearance in the periocular region. She presented no other lesions and there was no family history of interest. Skin biopsy revealed a capillary proliferation, negative for GLUT-1, consistent with a diagnosis of capillary malformation. Patient 2 was a girl aged 8 years, with no past history of interest. She was referred to our clinic for macules on the left side of her face. The macules had first been noticed by her parents when the child was 4 years old, and they had grown progressively. Her parents stated that the macules became more evident in hot environments and after physical exertion. On examination, patchy pink macules were observed in a segmental distribution along the left body of the jaw and they were seen to become more intense after the patient performed physical exercise. Patient 3 was a 7-year-old boy with no past history of interest. He was seen for a lesion in the right jaw region. His parents had first noticed the lesion when the child was about 4 years old. They stated that it had been asymptomatic, although its intensity increased with physical activity