Angioma serpiginosum: report of two unusual cases

Abstract

Angioma serpiginosum (AS) is a rare vascular naevoid disorder due to ectatic dilation of capillaries in the papillary dermis. The condition is found almost exclusively in females who have grouped erythematous punctate lesions usually on the lower limbs or buttocks. We report two rare cases of AS, each representing a different and unusual clinical feature of AS: one with familial involvement and the other with an extensive distribution of lesions.

Angioma Serpiginosum Arranged in a Systematized Segmental Pattern Suggesting Mosaicism

“…However, it is difficult to categorize this disorder according to the foregoing classification of mosaic patterns. A similar asymmetric arrangement of AS lesions has been noted by some previous authors [5,6,7]. On the other hand, it remains conceivable that in some other previous case reports, the AS lesions followed the lines of Blaschko [8, 9].…”

“…As initially noted by Hutchinson [1], it appears most frequently in adolescent females, with the legs being the areas of predilection. The disorder is often arranged in an asymmetric or otherwise segmental pattern [3,4,5,6] that may also involve the trunk [5, 7]. Sometimes, the distribution of lesions may even be reminiscent of the lines of Blaschko [8, 9].…”

Angioma serpiginosum with oesophageal papillomatosis is an X-linked dominant condition that maps to Xp11.3–Xq12

“…Extensive affection of the skin is rare. 4,12,16 The palms and soles are not affected, nor is the oral mucosa.…”

“…15 Familial occurrence is rare: there is no male-to-male transmission reported but two father-to-daughter transmissions are known. 11,12 In the only large family with angioma serpiginosum so far described, four out of six siblings were affected (two brothers and two sisters), and three of them had an affected daughter, suggesting autosomal dominant inheritance. 11 We have investigated a Norwegian family with suspected X-linked dominant angioma serpiginosum, the second family ever described with this usually sporadic condition.…”

“…9,10 Only incomplete blanching by pressure can be obtained. 11,12 The rash is otherwise asymptomatic, and the patients' problems are mainly of cosmetic nature. Treatment by pulsed dye laser can be successful.…”