Infantile pyramidal protrusion as a manifestation of lichen sclerosus et atrophicus

Background: A perineal infantile lesion previously described as "skin tag/fold" has recently been named infantile perianal pyramidal protrusion. It appears on the perineal median raphe of girls as a pyramidal soft tissue swelling, covered by smooth, red or rose-colored skin. Its pathogenesis is unknown. As in the case of other perianal lesions, knowledge about it is important, as concern about signs of child abuse grows. Observations: Four girls, 2 of them sisters, with infantile perianal pyramidal protrusion were studied. Three of these girls showed subtle clinical evidence of classic lichen sclerosus et atrophicus on first examination. The other girl developed vulvar lesions of lichen sclerosus et atrophicus months after the diagnosis of infantile perianal pyramidal protrusion. All 4 protrusions disclosed histopathological findings diagnostic of lichen sclerosus et atrophicus. Conclusions: Infantile perianal pyramidal protrusion is, at least in some patients, a peculiar form of lichen sclerosus et atrophicus that can precede other, more characteristic manifestations. We suggest changing the name to the more precise infantile perineal protrusion. Knowledge of this hitherto unrecognized clinical form of lichen sclerosus el atrophicus can help to explain anogenital symptoms and avoid its misinterpretation as a sign of sexual abuse.