Angiokeratoma‐like changes in extragenital and genital lichen sclerosus

Abstract

Hemorrhagic blisters have rarely been described developing in the background of either genital or extragenital lichen sclerosus and have invariably been designated clinically as telangiectatic, hemorrhagic or bullous lichen sclerosus. We describe three patients with extragenital and genital lichen sclerosus, who presented clinically with hemorrhagic plaques and/or papules. In addition to the classical histology of lichen sclerosus, dilated, congested and focally thrombosed vascular channels lined by flat endothelium were seen within the sclerotic dermal collagen. They were in close proximity to and even in contact with the overlying epidermis and thus mimicked an angiokeratoma. Angiokeratoma-like changes in lichen sclerosus represent secondary features because of damage to the dermis by lichen sclerosus and are characterized histologically by ectatic thin-walled vascular spaces in the papillary dermis intimately associated with the epidermis. Increased venous pressure, local trauma, degenerative changes in the elastic tissue of the vessel wall and/or surrounding supportive tissue, as well as abnormalities in the extracellular protein network, appear to be implicated in their pathogenesis.

Extragenital Bullous Lichen Sclerosus in a Pediatric Patient: A Case Report and Literature Review

“…Thus, according to the Japanese literature, blister formation is observed more frequently in the extragenital region . This finding has not been clearly established in the English literature . Nonetheless, the occurrence of hemorrhagic bullae in extragenital lichen sclerosus is gaining recognition, and cases such as ours should raise clinical awareness of the probable association between extragenital lichen sclerosus and bullous formation.…”

“…The most common site for these lesions is the trunk, followed by the umbilical region, neck, axillae, and wrists. Whereas women are affected 10 times more than men in the classic genital form, the female‐to‐male ratio for extragenital lichen sclerosus is 1:1 . Exclusive extragenital involvement is rare and is estimated to be approximately 2.5% of cases of lichen sclerosus .…”

“…Variations from the classic porcelain‐white plaques of lichen sclerosus occur and can complicate diagnosis. Hemorrhagic blisters in the setting of genital or extragenital lichen sclerosus have been described in the adult literature and have been recognized clinically as bullous lichen sclerosus . We present an unprecedented case of extragenital bullous lichen sclerosus in a child.…”

Lichen sclerosis: clinicopathological study of 60 cases from Lebanon

“…Furthermore, AK‐like changes were observed in two (3.1%) genital LS cases. This change, which has been rarely reported in association with LS, is probably secondary to multiple factors including damage to dermal supportive connective tissue characteristic of LS, increased venous pressure, and local trauma. Similar factors may also contribute to the development of milia in association with LS, as was seen in one of our LS cases.…”

“…Compared to extragenital LS, genital LS may additionally exhibit spongiotic dermatitis changes and epidermal hyperplasia at times severe enough to be pseudoepitheliomatous . Additional uncommon or rare microscopic features reported with LS include the presence of eosinophils, angiokeratoma (AK)‐like changes, epidermotropic lymphocytes mimicking mycosis fungoides (MF)/cutaneous T‐cell lymphoma, and milia formation …”