Postzygotic mosaicism in a woman with Goltz syndrome mimics segmental angioma serpiginosum

Angioma‐serpiginosum‐like and hyperkeratotic lesions in a patient with Goltz syndrome

“…When biopsied, they usually show an increase in dermal collagen, 10 with thickened collagen bundles, and loss of adnexal structures . 13 The overlying epidermis is acanthotic and hyperkeratotic, 17 and sometimes parakeratotic, 19 as in our case. In some cases, a dermal inflammatory infiltrate accompanies fibrosis of the superficial dermis.…”

“…Angioma‐serpiginosum‐like changes, like the ones seen in our biopsy, are also useful in the absence of dermal hypoplasia 14‐17 . The cutaneous vascular abnormalities seen in Goltz syndrome are clinically evidenced as telangiectasias.…”

“…Some examples of Goltz syndrome develop cutaneous hyperkeratotic lesions, which have also very occasionally been histopathologically described in the literature. When biopsied, they usually show an increase in dermal collagen, 10 with thickened collagen bundles, and loss of adnexal structures .13 The overlying epidermis is acanthotic and hyperkeratotic, 17 and sometimes parakeratotic, 19 as in our case. In some cases, a dermal inflammatory infiltrate accompanies fibrosis of the superficial dermis 16 .…”

“…Angioma-serpiginosum-like changes, like the ones seen in our biopsy, are also useful in the absence of dermal hypoplasia. [14][15][16][17] The cutaneous vascular abnormalities seen in Goltz syndrome are clinically evidenced as telangiectasias. The biopsy shows multiple dilated, thin-walled, small blood vessels in the papillary dermis without signs of vasculitis or extravasation of erythrocytes.…”