Novel Clinical Observations on Benign Cephalic Histiocytosis in a Large Series

Abstract

With 11 additional cases from a single center, BCH seems to be an underrecognized disease. Its clinical presentation is not uniform. Considering that most of the patients in this series and those previously reported had extracephalic involvement, the term "cephalic" needs to be reevaluated.

Benign cephalic histiocytosis: a case report of unusual presentation with initial appearance of extrafacial lesions

“…Benign cephalic histiocytosis (BCH) belongs to the non‐Langerhans cell histiocytosis family. It is a rare disease, and approximately 70 cases have been reported since its first description in 1971 by Gianotti, Caputo, and Ermacora 1,2 . Herein we present a case with clinical features that diverge from the majority of previous records, with initial lesions on extrafacial sites.…”

“…Benign cephalic histiocytosis is a rare disease first described by Gianotti et al in 1971 1 . To date, there are approximately 70 cases reported, of which only four had initial lesions in extrafacial sites 2–4 . The disease is more prevalent in males.…”

“…The mean age of patients at diagnosis is 7 months, with up to 50% of cases occurring before 6 months of age 4 . BCH is considered a localized cutaneous disease, with no systemic or mucosal involvement, although two previous cases associated with diabetes mellitus and diabetes insipidus have been reported 2 . Lesions usually appear up to 3 years of age, with 65% located in the upper half of the body, whereby the face represents the main site of involvement 2,4 …”

“…Lesions are clinically characterized by asymptomatic erythematous, yellowish or brownish macules or papules of 2–8 mm. Moreover, lesions are self‐limited, with complete regression varying from 9 months to 9 years from the initial appearance 2,4,5 . The etiology and pathophysiology are unknown.…”

“…The etiology and pathophysiology are unknown. BCH may belong to a spectrum of non‐Langerhans cell histiocytosis with clinical, histological, and ultrastructural overlapping between juvenile xanthogranuloma and generalized eruptive xanthoma 2,4,5 . Histologically, there is histiocytic cell proliferation in the superficial to mid‐reticular dermis.…”