Infantile perianal pyramidal protrusion is an important, relatively newly recognized condition, which is thought to be under-reported in the pediatric literature. Previous reports refer to these lesions as skin tags or skin folds. More recently, the name infantile perianal pyramidal protrusion has been suggested, based on the characteristic presentation of the lesion in prepubertal children, especially girls. Recognition of these lesions has many implications regarding proper diagnosis and management. They may easily be mistaken for condyloma accuminata or as a sign of trauma, leading to an investigation of sexual abuse. This situation may be avoided with awareness of this entity in combination with thorough history taking and a classic presentation. We present a classic presentation of infantile perianal pyramidal protrusion and further discuss this condition.
Her developmental milestones were normal. A skin biopsy specimen showed marked hyperkeratosis, hypergranulosis, acanthosis, and papillomatosis -suggestive of verrucous epidermal nevus.A 21-year-old man had multiple linear verrucous papules and plaques with minimal scaling over the left half of the body, present since early childhood. The lesions extended onto the glans penis and scrotum. A biopsy specimen taken from the glans revealed hyperkeratosis, acanthosis, spongiosis, and a mild upper dermal lymphocytic infiltrate -features suggestive of ILVEN.These three patients had involvement of the soles or genitalia or both. In the second patient, the lesions were confined to the left sole. Although inguinogenital involvement is rare in patients with VEN, it is not uncommon in the setting of ILVEN. Rogers et al (3), in their series of 233 patients with epidermal nevi and/or the epidermal nevus syndromes, described ILVEN in eight and of these patients, three had inguinogenital involvement. Altman and Mehregan (4) as well, in their review of 25 patients with ILVEN, described lesions in the inguinogenital area in a few. Epidermal nevus can involve the palms and soles but is rare in those sites. None of the patients in the above mentioned series had involvement of the palms or soles. Incomplete reporting might contribute to the apparent low frequency of volar skin involvement in VEN and ILVEN.
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