Benign Cephalic Histiocytosis: Case Report and Review of the Literature

Abstract

Benign cephalic histiocytosis (BCH) is a rare type of non-Langerhans histiocytic disorder, usually presenting with small, yellow-red or yellow-brown, asymptomatic papules, located mostly on the head and neck of infants and young children. The histopathologic hallmark of BCH is a well-circumscribed histiocytic infiltrate in the superficial to mid-reticular dermis. BCH is a self-healing disorder, with the eruptions regressing spontaneously in most cases; therefore no treatment is required. We present a case of BCH in a 7-month-old boy who was referred to our department with multiple, asymptomatic, yellow-red papules located on his face, and we review 55 cases published in the English-language literature in an effort to better describe and understand this unusual entity.

Increasing number of yellowish papules on the face

“…The histopathologic hallmark is a well‐circumscribed histiocytic infiltrate in the superficial to mid reticular dermis. Immunohistochemical profile demonstrates positive staining for factor XIIIa, fascin and CD68, but negative for CD1a, langerin and S100 . To date, only about 60 cases have been described.…”

“…To date, only about 60 cases have been described. The disorder typically begins by the age of 1 year and always within the first 3 years of life . Clinically, it is characterised by yellow to red‐brown macules and papules, initially on the face, with subsequent appearance on the ears, neck and, occasionally, lesions may develop on the trunk and arms.…”

Treatment of benign cephalic histiocytosis with topical 1% rapamycin ointment

“…To our knowledge, this is the first reported case of topical rapamycin used to treat BCH. A potential limitation of our case is the spontaneously remitting nature of BCH, which could create a challenge in assessing treatment response. However, our split‐face model helped illustrate the difference in resolution rate between the treated and nontreated sides.…”

Novel Clinical Observations on Benign Cephalic Histiocytosis in a Large Series

“…All lesions spontaneously resolve in a few years without treatment. Patsatsi et al found in 2014 that there had been only 55 reported cases in the English‐language literature since Gianotti et al first described this disorder in 1971. With a few additional cases thereafter, we presume that there have now been nearly 60 reported cases .…”