Peer Reviewed


Benign Cephalic Histiocytosis in an Infant

Brooks David Kimmis, MD1 • Kristi Canty, MD2

1Division of Dermatology, University of Kansas School of Medicine, Kansas City, Kansas
2Department of Dermatology, Children’s Mercy Hospital, Clinics on Broadway, Kansas City, Missouri

Kimmis BD, Canty K. Benign cephalic histiocytosis in an infant. Consultant. Published online October 28, 2021. doi:10.25270/con.2021.10.00013

Received April 10, 2021. Accepted May 25, 2021.

The authors report no relevant financial relationships.

Brooks David Kimmis, MD, University of Kansas School of Medicine, 3901 Rainbow Boulevard, Kansas City, KS 66160 (


A 17-month-old boy was referred to our pediatric dermatology clinic for evaluation of a facial rash that had been present since age 6 months. The patient’s mother reported that the eruption had initially started as papules on the right cheek and subsequently spread to the left (Figure 1), with new papules appearing gradually over time.

Figure 1. Patient’s face with scattered rash.
Figure 1.
Patient’s face with scattered rash.


The eruption was asymptomatic, unchanged with daily moisturizers, and seemed to become more noticeable when the patient was irritated or crying. There was no family history of similar skin eruptions.

Physical examination. Upon examination, multiple pink-orange, dermal papules with increased vascularity were seen on the bilateral cheeks, forehead, and left upper eyelid. The patient’s trunk, extremities, oral mucosa, and acral surfaces were spared.

A punch biopsy of a papule on the left lateral cheek revealed an infiltrate of histiocytes and a few eosinophils in the superficial dermis (Figures 2 and 3). Clinical and histopathologic findings were consistent with a diagnosis of benign cephalic histiocytosis.

 Figure 2. Results of a skin biopsy showed increased histiocytes in the papillary dermis (hematoxylin and eosin stain at 200× magnification).
Figure 2. Results of a skin biopsy showed increased histiocytes in the papillary dermis (hematoxylin and eosin stain at 200× magnification).

Figure 3. The histiocytes were diffusely positive for CD68 (CD68 Immunohistochemical stain, 200× magnification).
Figure 3. The histiocytes were diffusely positive for CD68 (CD68 Immunohistochemical stain, 200× magnification).


Discussion. First described in 1971 by Gianotti and colleagues, benign cephalic histiocytosis, or papillary histiocytosis of the head, is a rare variant of non-Langerhans cell histiocytosis, typically occurring in infants and young children.1 The average age of onset is 15 months, and there exists an equal distribution among boys and girls.2

Benign cephalic histiocytosis presents as asymptomatic, small, yellow to red-brown macules and papules located on the head and neck. Lesions can spread to the trunk and extremities, and one study suggests the eruption may involve noncephalic skin more often than previously thought.3 The disorder is limited to the skin, sparing the mucosal membranes and internal organs. The eruption resolves spontaneously over an average of 50 months, sometimes leaving behind hyperpigmentation or small, atrophic scarring.2,4 Although benign cephalic histiocytosis is not thought to have systemic involvement, 2 reports of coexisting systemic illness, with diabetes mellitus and diabetes insipidus in separate instances, have been reported.5,6 However, these associations were thought to be coincidental.

The diagnosis is mostly clinical but should be confirmed with histopathology and immunohistochemical staining.4 Histology demonstrated a histiocytic infiltrate in the superficial to midreticular dermis with characteristic staining pattern.2-4 The histiocytes stain positive for factor XIIIa, HAM-56, fascin, and CD68 and stain negative for CD1a, langerin, and S100.7-9

Benign cephalic histiocytosis can be mistaken for juvenile xanthogranluoma, which was considered in our patient’s case. Juvenile xanthogranluoma is often more widespread and can have extracutaneous involvement, specifically ocular, which is not seen in patients with benign cephalic histiocytosis.2-4 Benign cephalic histiocytosis can also be distinguished from juvenile xanthogranluoma on histology.

Generalized eruptive histiocytosis and Langerhans cell histiocytosis should also be considered in the differential diagnosis. Generalized eruptive histiocytosis is histologically similar to benign cephalic histiocytosis but is seen mostly in adults and can have mucosal involvement.2-4 Langerhans cell histiocytosis differs from benign cephalic histiocytosis with a scaled, crusted, or sometimes petechial eruption often on flexor, scalp, or intertriginous surfaces and has the possibility of visceral involvement.2 Histology and immunostaining of Langerhans cell histiocytosis is distinctive.2,8,9

Conclusions. Benign cephalic histiocytosis is a rare disease with an estimated 60 cases reported in the English-language literature.3,4 Despite its rarity, benign cephalic histiocytosis is an important disease to recognize when considering histiocytic skin lesions or other mimickers. Given its extremely low prevalence, it is our hope that sharing this case assists clinicians in recognizing benign cephalic histiocytosis and avoid unnecessary, aggressive treatment or workup for this self-limiting, benign condition.


1. Gianotti F, Caputo R, Ermacora E. Singular infantile histiocytosis with cells with intracytoplasmic vermiform particles. Bull Soc Fr Dermatol Syphiligr. 1971;78(3):232-233.

2. Jih DM, Salcedo SL, Jaworsky C. Benign cephalic histiocytosis: a case report and review. J Am Acad Dermatol. 2002;47(6):908-913.

3. Polat Ekinci A, Buyukbabani N, Baykal C. Novel clinical observations on benign cephalic histiocytosis in a large series. Pediatr Dermatol. 2017;34(4):392-397.

4. Patsatsi A, Kyriakou A, Sotiriadis D. Benign cephalic histiocytosis: case report and review of the literature. Pediatr Dermatol. 2014;31(5):547-550.

5. Weston WL, Travers SH, Mierau GW, Heasley D, Fitzpatrick J. Benign cephalic histiocytosis with diabetes insipidus. Pediatr Dermatol. 2000;17(4):296-298.

6. Saez-De-Ocariz M, Lopez-Corella E, Duran-McKinster C, Orozco-Covarrubias L, Ruiz-Maldonado R. Benign cephalic histiocytosis preceding the development of insulin-dependent diabetes mellitus. Pediatr Dermatol. 2006;23(1):101-102.

7. D'Auria AA, De Clerck B, Kim G. Benign cephalic histiocytosis with S-100 protein positivity. J Cutan Pathol. 2011;38(10):842-843.

8. Mitsui Y, Ogawa K, Mashiba K, Fukumoto T, Asada H. Case of S100-positive benign cephalic histiocytosis involving monocyte/macrophage lineage marker expression. J Dermatol. 2018;45(12):e344-e345.

9. Hattori-Murakami M, Tanaka R, Yamamoto T, Hayashi H, Nishimura H, Fujimoto W. Benign cephalic histiocytosis: a case with infiltration of CD1a-positive langerin-negative cells. J Dermatol. 2018;45(7):e181-e182.