|LETTER TO EDITOR
|Year : 2015 | Volume
| Issue : 4 | Page : 274-275
Benign cephalic histiocytosis
Srinath M Kambil, Ravikant Chauhan, Ramesh M Bhat, Sukumar Dandekeri
Department of Dermatology, Father Muller Medical College, Kankanady, Mangalore, Karnataka, India
|Date of Web Publication||24-Sep-2015|
Srinath M Kambil
Department of Dermatology, Father Muller Medical College, Kankanady, Mangalore - 575 002, Karnataka
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Kambil SM, Chauhan R, Bhat RM, Dandekeri S. Benign cephalic histiocytosis
. Indian J Paediatr Dermatol 2015;16:274-5
Benign cephalic histiocytosis (BCH) is a rare, benign, nonlangerhan cell histiocytosis (LCH) seen in infants and young children. It presents as a self-healing eruption of red-brown papules on head and neck initially and lesions may subsequently spread to involve trunk, arms, and legs. Histological finding of CD68 and factor XIIIa positive histiocytes in the dermis helps to confirm the diagnosis. 
An 18-month-old boy presented with multiple discrete brown papules and macules distributed over the cheeks, ears, chin, and bilateral upper arms [Figure 1]a and b]. Few hyperpigmented macules were seen over the trunk, bilateral forearms, and thighs. Lesions first appeared on the cheeks when the child was 6 months of age and spread gradually to involve other areas. There was no associated history of bone pain, increased urination or other systemic complaints. Mucosa, palms, and soles were spared and Darier's sign was negative. There was no hepatosplenomegaly and lymphadenopathy. Skeletal, neurodevelopmental, and ophthalmological examination were normal. Routine blood investigations including serum lipid profile and urine analysis were normal. We considered the differential diagnosis of BCH, generalized eruptive histiocytosis (GEH), juvenile xanthogranuloma, mastocytosis, and langerhan cell histiocytosis. Histological examination showed unremarkable epidermis and superficial dermis showed perivascular histiocytic infiltrate without epidermotrophism. Foamy cells and giant cells were not seen [Figure 2]. In the immunohistochemistry analysis, the cells stained positive for CD68, [Figure 3] but negative for CD1a and S100 [Figure 4]a and b]. A final diagnosis of BCH was made with clinical, histopathological and immunohistological correlation.
|Figure 1: (a and b) Brown papules and macules seen over face and upper arm|
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|Figure 2: (a and b) Superficial dermis showed perivascular histiocytic infiltrate without epidermotrophism|
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|Figure 4: (a and b) Lesional cells showing negative staining with CD1a and S100|
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Benign cephalic histiocytosis was first described by Gianotti, Caputo, and Ermacora in 1971. There have been 56 cases of BCH reported in literature so far. A slight male preponderance (1.67:1) has been seen and in 50% of cases lesions start developing by 6 months of age. , BCH typically presents as yellow to red-brown papules of 2-8 mm size located on the head, neck, and shoulders. Lesions may subsequently spread to involve trunk arms and legs, but spares the palms, soles, mucosa, eyes, and internal organs. Though single case reports of diabetes insipidus and insulin-dependent diabetes mellitus associated with BCH have been reported, systemic involvement is not usually seen. , Histological examination shows circumscribed histiocytic infiltrate in the upper dermis, but foamy cells and touton giant cells are usually absent. Comma-shaped or worm-like bodies are seen in 5-30% of histiocytes on electron microscopy, but birbeck granules are absent in BCH.  Immunohistologically the lesional cells are positively for CD68, factor XIIIa, and fascin and negative for CD1a, S100, and langerin. BCH must be differentiated from histiocytic disorders like juvenile xanthogranuloma, GEH, LCH, and non histiocytic disorders like mastocytosis, plane warts, syringoma, and spitz nevi. Lesions of BCH spontaneously regress in an average of 50 months and may sometimes leave behind hyperpigmented macules or atrophic scars. No treatment is required and the benign nature of BCH should be explained to the parents of the child.
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| References|| |
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]