Novel Clinical Observations on Benign Cephalic Histiocytosis in a Large Series

Benign cephalic histiocytosis (BCH) is a rare form of non-Langerhans cell histiocytosis in infants and young children characterized by self-healing macules and papules occurring primarily in the head and neck region. So far there have been nearly 60 reported cases in the English-language literature.

In this retrospective study, we evaluated clinical features and follow-up data of 11 patients diagnosed with BCH between 2004 and 2016 in the Department of Dermatovenereology, Istanbul Medical Faculty.

There were 5 girls and 6 boys (median age 24 months, range 9-72 months). The median age at the onset of lesions was 8 months (range 3-36 months). The lesions first appeared on the face in 10 patients and on the trunk in 1. Proximal parts of the extremities and trunk were also involved in nine patients (81.8%). Patients were categorized into two groups based on their clinical features; five had 20 to 30 predominantly red-brown dome-shaped papules and six had 50 to hundreds of yellow-brown or predominantly pinkish brown flat papules. Four patients were lost to follow-up. In seven patients with a mean follow-up of 5 years, four had nearly complete resolution and three showed remarkable regression without treatment.

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.