Angioma serpiginosum, eine Hautver�nderung entlang den Blaschko-Linien?

Abstract

We present a 34-year-old woman with angioma serpiginosum on the medial aspect of the left thigh, extending to the groin and the lower abdomen, and on the volar aspect of the left forearm. In addition to the late onset, the distribution along the lines of Blaschko was a remarkable feature of this case. We therefore reexamined our recent cases of angioma serpiginosum. In 6 of 7 cases, the distribution indeed corresponded to the lines of Blaschko. Thus, we suggest adding angioma serpiginosum to the list of skin lesions that may follow the lines of Blaschko.

Angioma Serpiginosum Arranged in a Systematized Segmental Pattern Suggesting Mosaicism

“…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]. …”

“…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]. A familial occurrence of AS has also been observed and, remarkably, all of the affected family members showed a rather symmetric and nonsegmental involvement [10].…”

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

“…In the only other large family with angioma serpiginosum reported so far, the pattern of inheritance is best compatible with autosomal dominant inheritance but does not exclude X-linked dominant inheritance. 11 As most patients from the literature are female, and the vascular streaks often follow Blaschko's lines, 3,15 an X-linked dominant acting gene is likely to be the major cause in most sporadic cases. The few described males often have segmental affection, 10 suggesting somatic mutations.…”

“…2 The rash has a serpiginous or gyrate pattern that follows Blaschko's dermal migration lines. 3 The rash resembles purpura, 4 -6 but there are no signs of inflammation or bleeding. 7,8 The vascular capillary nests in the subepidermal papillae are not simple telangiectasias but represent a capillary naevus with both dilatation and proliferation of thick-walled capillaries having slit-like protrusions of capillary lumen into the endothelial lining.…”

Reply to Happle

“…3 Furthermore, in several patients affected skin following Blaschko lines has been described, 4 and no male to male transmission has been reported. Indeed, OMIM mentions that the few male cases with AS that have been described 'may actually represent somatic mosaicism of an X-linked gene'.…”