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Year : 2021  |  Volume : 22  |  Issue : 1  |  Page : 85-86

Benign Cephalic Histiocytosis with Unique Dermoscopic Pattern

1 Department of Dermatology and Venereology, Government Medical College, Kozhikode, Kerala, India
2 Department of Pathology, Government Medical College, Kozhikode, Kerala, India

Date of Submission24-Oct-2018
Date of Decision27-Dec-2018
Date of Acceptance14-Jun-2020
Date of Web Publication31-Dec-2020

Correspondence Address:
Anza Khader
5/1986 B, “Maskan” Rajiv Nagar Colony, P. O. Puthiyara, Kozhikode - 673 004, Kerala
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijpd.IJPD_122_18

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How to cite this article:
Sasidharanpillai S, Khader A, Govindan A, Paul N, John N. Benign Cephalic Histiocytosis with Unique Dermoscopic Pattern. Indian J Paediatr Dermatol 2021;22:85-6

How to cite this URL:
Sasidharanpillai S, Khader A, Govindan A, Paul N, John N. Benign Cephalic Histiocytosis with Unique Dermoscopic Pattern. Indian J Paediatr Dermatol [serial online] 2021 [cited 2021 Apr 20];22:85-6. Available from: https://www.ijpd.in/text.asp?2021/22/1/85/305799


Histiocytic skin diseases are classified as Langerhans' cell histiocytosis (LCH) or non-LCH, based on the pathology of the proliferating histiocytes. The main types of non-LCH in children are benign cephalic histiocytosis (BCH), juvenile xanthogranuloma (JXG), xanthoma disseminatum, and generalized eruptive histiocytosis. BCH is a rare, benign, non-LCH seen in infants and young children. Association with diabetes insipidus has been reported, but is extremely rare. All lesions spontaneously resolve in a few years without treatment leaving atrophied pigmented macules.[1],[2]

A 11-month-old male baby, first-order child of a nonconsanguineous marriage, presented with a history of asymptomatic raised lesions of 1-month duration, initially over the face which gradually progressed to involve the arms. There was no history of associated systemic symptoms. On examination, he had multiple, discrete, skin colored to reddish brown papules, involving the lower eyelids, face, and both arms [Figure 1]. Darier's sign was negative. There was no hepatomegaly, lymphadenopathy, skeletal abnormalities, or any features of involvement of other systems. Neurological and ophthalmological evaluation was normal. We proceeded with dermoscopy to detect any characteristic finding. Dermoscopy on nonpolarized light showed papule with central depression and on polarized light revealed yellow clods (yellow arrow), red dots (black arrow), red structureless areas (white arrow), and brown black v-shaped arcuates (red arrow) arranged in a well-circumscribed manner simulating the cut surface of kiwi fruit [Figure 2]a and [Figure 2]b.
Figure 1: Multiple, discrete, skin colored to reddish brown papules, involving the lower eyelids and face

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Figure 2: (a) Dermoscopy on nonpolarized light showed papule with central depression, (b) dermoscopy on polarized light revealed cut kiwi fruit-like appearance

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Routine blood investigations, peripheral smear, ultrasonography of the abdomen, and chest X-ray were normal. Histopathological examination revealed focal epidermal atrophy, elongated rete ridges with increased pigmentation, circumscribed histiocytic infiltration of the upper dermis, and dilated capillaries [Figure 3]a and [Figure 3]b. Touton giant cells were not seen. On immunohistochemistry, these cells were CD1a and S100 negative and CD68 positive [Figure 4]a,[Figure 4]b. A final diagnosis of BCH was made depending on clinical, histological, and immunohistochemical findings.
Figure 3: (a) Diffuse upper dermal infiltrate of histiocytes (H and E, ×40), (b) histiocytes with pale cytoplasm and oval or elongate vesicular nuclei (H and E, ×400)

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Figure 4: (a) Histiocytes are CD1a negative (CD1a antibody ×400), (b) Histiocytes are negative for S-100 (S-100 antibody ×400)

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BCH was first described by Gianotti in 1971 as “infantile histiocytosis with cells with intracytoplasmic vermiform particles.” The disease occurs in infants and young children as asymptomatic macules and papules with varying colors ranging from yellow to red brown on the head-and-neck region, which subsequently progresses in a descending manner to involve the trunk, arms, and legs but sparing distal extremities. Mucous membranes and internal organs are never involved.[3] Our patient presented with asymptomatic reddish brown discrete papules over the face which later involved the arms. Although systemic associations such as diabetes insipidus or diabetes mellitus have been reported, our patient did not had any suggestive symptoms.

One of the differentials JXG mostly occurs in the 1st year of life as small yellowish brown cupuliform papules with telangiectasias on its surface and is distributed more widely.[2],[3]

Self-regressing cutaneous LCH may present with scaly or crusted erythematopurpuric papules over the face and trunk, characteristically sparing mucosa.[4]

The presentation of asymptomatic nontelangiectatic papules without scaling or crusting limited to the head, neck, and arms made possibility of BCH more likely.

Dermoscopy on nonpolarized light in our case showed papule with central depression and on polarized light revealed yellow clods, red dots, and brown black arcuates. The central depression correlates with the focal epidermal atrophy in the biopsy, yellow clods due to histiocytes in the dermis, red dots due to capillary congestion, and brown black arcuates as a result of elongated rete ridges and increased pigmentation.

Dermoscopy of JXG, which is clinically similar to BCH, shows central yellowish structureless areas and peripheral vascular pattern.[5] Spitz nevus, sebaceous hyperplasia, xanthomatous dermatofibroma, histiocytic sarcoma, and mastocytoma also show similar features on dermoscopy. Our case revealed yellow clods, vascular pattern, and pigmentary changes, although telangiectasia and pigmentation was not marked clinically. To the best of our knowledge, dermoscopy finding in BCH has not been reported in the literature.

Histologically, BCH is characterized by a diffuse infiltrate of histiocytes in the upper dermis in close apposition to the undersurface of the epidermis associated with a mixed inflammatory infiltrate of lymphocytes, rarely, eosinophils. The Langerhans cell markers CD1a and S-100 protein are negative, whereas the macrophage/histiocytic markers CD68 and factor XIIIa are positive.[6]

As spontaneous regression of BCH is known, our patient's parents were reassured, and the patient was kept under follow-up. On repeat visit, lesions were found to reduce in size and no new lesions erupted.

Our case highlights unique dermoscopic findings in BCH with histological correlation, which has not been reported in the literature. Dermoscopy function as a useful adjuvant in the diagnosis of this rare entity of BCH is in infants.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Koca R, Bektaş S, Altinyazar HC, Sezer T. Benign cephalic histiocytosis: A case report. Ann Dermatol 2011;23:508-11.  Back to cited text no. 1
Luz FB, Gaspar AP, Kalil-Gaspar N, Ramos-e-Silva M. Histiocytes and non-Langerhans cell histiocytoses in dermatology. An Bras Dermatol 2003;78:99-118.  Back to cited text no. 2
Daye M, Dogan S, Mevlitoglu I, Toy H. Benign cephalic histiocytosis. Indian J Dermatol Venereol Leprol 2013;79:713-4.  Back to cited text no. 3
[PUBMED]  [Full text]  
Battistella M, Fraitag S, Teillac DH, Brousse N, de Prost Y, Bodemer C. Neonatal and early infantile cutaneous langerhans cell histiocytosis: Comparison of self-regressive and non-self-regressive forms. Arch Dermatol 2010;146:149-56.  Back to cited text no. 4
Jo G, Mun J. Is the setting sun dermoscopic pattern specific to juvenile xanthogranuloma? J Am Acad Dermatol 2018;78(2):e 51-2.  Back to cited text no. 5
Kambil SM, Chauhan R, Bhat RM, Dandekeri S. Benign cephalic histiocytosis. Indian J Paediatr Dermatol 2015;16:274-5.  Back to cited text no. 6
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  [Figure 1], [Figure 2], [Figure 3], [Figure 4]


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