Indian Journal of Dermatology
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Year : 2013  |  Volume : 58  |  Issue : 5  |  Page : 411
A case of Juvenile xanthogranuloma in the nasal cavity of a neonate

Department of Dermatology, Eulji Medical Center, College of Medicine, Eulji University, Seoul, Korea

Date of Web Publication30-Aug-2013

Correspondence Address:
Seo Wan Kim
Department of Dermatology, Eulji Medical Center, College of Medicine, Eulji University, Seoul
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0019-5154.117364

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How to cite this article:
Kim SW, Han TY, Lee JH, Son SJ. A case of Juvenile xanthogranuloma in the nasal cavity of a neonate. Indian J Dermatol 2013;58:411

How to cite this URL:
Kim SW, Han TY, Lee JH, Son SJ. A case of Juvenile xanthogranuloma in the nasal cavity of a neonate. Indian J Dermatol [serial online] 2013 [cited 2020 Jul 14];58:411. Available from:


Juvenile xanthogranuloma (JXG) is a benign, self-limited lesion that is composed of histiocyte proliferation. It most commonly develops on the head and neck, followed by the upper extremities and the lower extremities, but rarely develops in the nasal cavity.

An 8-day-old girl presented with a nasal tumor at birth. Physical examination demonstrated a solitary yellowish-to-erythematous, arc-shaped, about 1 cm-sized hard nodule on the nasal septum and the base of both nasal vestibules [Figure 1]a. A punch biopsy specimen of the lesion showed dense intradermal histiocytic infiltrates composed of a mixed population of foamy macrophages, lymphocytes and foreign body giant cells, but there were no Touton giant cells [Figure 2]a and b. Immunohistochemical studies showed reactivity for CD68, whereas the proliferating cells were negative for S-100 protein. Histopathological findings were consistent with juvenile xanthogranuloma. We have made periodic observations, and the lesion showed spontaneous regression after 6 months, and there has been no recurrence and complications after 12 months follow-up [Figure 1]b.
Figure 1: (a) A solitary yellowish - to - erythematous arc - shaped about 1 cm-sized hard nodule on the nasal septum and at the base of both nasal vestibules. (b) After 6 months, the lesion regressed spontaneously

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Figure 2: (a) Dense histiocytic infiltrate in the dermis (H and E, ×100). (b) Mixed cell infiltration of foamy macrophages, lymphocytes and foreign body giant cells (H and E, ×400)

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JXG is the most common form of non-Langerhans cell histiocytosis and is considered a benign histiocytic proliferation. It occurs predominantly in infancy and childhood and regress spontaneously. It presents as a well-demarcated, firm, round or oval-shaped, yellowish to red-brown colored nodule, and most cases develop as single lesions.

The most commonly involved sites are the head and neck, followed by the trunk, upper extremities and lower extremities. [1] Five percent of JXG involve extracutaneous areas, and the most frequently involved extracutaneous area is the eye. [2] The nasal cavity is an uncommon site of presentation, where only six cases of xanthogranuloma of the nasal cavity have been reported in the English literature.

Histopathologically, JXG presents as an ill-defined, unencapsulated, dense histiocytic infiltrate in the papillary and reticular dermis. Neutrophils, eosinophils and lymphocytes are observed scattered within the lesion. The histopathologic hallmark in the mature lesion is the Touton giant cell, which is characterized by wreaths of multiple nuclei surrounding a glassy eosinophilic cytoplasm. In early JXG lesions, Touton giant cells may be either absent or sparse, as in the present case. [3] Immunohistochemical stains are typically positive for macrophage markers, such as CD68. Stains are typically negative for S-100 protein and CD1a, which are markers for Langerhans cells. [3] However, some cases of JXG showing positive immunoreactivity for the S-100 protein have been reported. [4] Consequently, S-100 reactivity cannot be the definitive markers for differentiating JXG from Langerhans cell histiocytosis.

The treatment of JXG depends on its size and clinical symptoms. Cutaneous lesions may be asymptomatic, and tends to be self-limited; therefore, no particular intervention is usually necessary. Sometimes, treatment may be required for extracutaneous lesions if JXG lesions cause significant symptoms, such as those involving the orbit. In case of those occurring in the nasal cavity, sometimes, nasal obstruction can develop when the size of the tumor progresses continuously. [5] Surgical excision is suggested for treatment, while some authors suggest that topical injection of steroid may be effective in the treatment of mucosal lesions. [6]

In conclusion, the clinical diagnosis of JXG is easily made. However, when it occurs in an unusual site, a dermatologist should consider JXG and perform histopathological examination to differentiate JXG from other diseases. We present a rare JXG in the nasal cavity in an 8-day-old girl, which was diagnosed based on its characteristic histological findings, and regressed spontaneously.

   References Top

1.Tahan SR, Paster-Levy C, Bhan K, Mihm MC. Juvenile xanthogranuloma; clinical and pathologic characterization. Arch Pathol Lab Med 1989;113:1057-61.  Back to cited text no. 1
2.Fang TJ, Lin CZ, Li HY. Juvenile xanthogranuloma of the nasal cavity. Int J Ped Otorhinolaryngol 2003;67:297-9.  Back to cited text no. 2
3.Chung BJ, Embi CS, Sellheyer K, Krakovitz PR. Pathology quiz case. Nasal juvenile xanthogranuloma (JXG). Arch Otolaryngol Head Neck Surg 2005;131:81-2.  Back to cited text no. 3
4.Yamamoto Y, Kadota M, Nishimura Y. A case of S-100-positive juvenile xanthogranuloma: A longitudinal observation. Pediatr Dermatol 2009;26:475-6.  Back to cited text no. 4
5.Saravanappa N, Rashid AM, Thebe PR, Davis JP. Juvenile xanthogranuloma of the nasal cavity. J Laryngol Otol 2000;114:460-1.  Back to cited text no. 5
6.Casteels I, Olver J, Malone M, Taylor D. Early treatment of juvenile xanthogranuloma of the iris with subconjunctival steroids. Br J Ophthalmol 1993;77:57-60.  Back to cited text no. 6


  [Figure 1], [Figure 2]


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