A 6-year-old male child presented with a raised lesion over scalp which the parents noticed one year back. There were no associated complaints. On examination, we found a single, firm, reddish-orange nodule of approximately 1 cm × 1 cm with overlying telangiectasias [Figure 1]. Dermoscopic examination of the nodule using DermLite DL4 (3Gen, San Juan Capistrano, California, USA) was done and images were captured with DermLite adapter for iPhone X. Dermoscopy revealed multiple, well-focused polymorphous vessels, namely linear-irregular, looped, and branching vessels at center as well as periphery with an underlying diffuse orangish-yellow background surrounded by erythematous zone [Figure 2]. Excision biopsy of the nodule was done and sent for histopathological examination with clinical differentials of juvenile xanthogranuloma (JXG), epidermal cyst, and trichilemmal cyst. Histopathological examination revealed a normal epidermis with poorly circumscribed collection of lymphocytes, foamy histiocytes, neutrophils, and giant cells. On higher power, multiple Touton and foreign body-type giant cells were seen in dermis [Figure 3a and b]. Diagnosis of JXG was made after clinical, dermoscopic and histopathological correlation.
Figure 1.
Single, reddish-orange nodule with overlying telangiectasia over scalp
Figure 2.
Multiple, polymorphous vessels over a yellowish-orange background surrounded by erythema (DermLite DL4, 10×, polarized mode, 3Gen, San Juan Capistrano, California)
Figure 3.
(a) Histopathological examination showing a normal epidermis with poorly circumscribed collection of lymphocytes, histiocytes, and giant cells in dermis (H&E, 4×). (b) Collection of foamy histiocytes and multiple Touton giant cells are seen in the dermis (H&E, 10×)
JXG is a disorder of histiocytic proliferation, having a benign self-limited course. It presents congenitally or during early childhood as yellowish-nodules, papules, or plaque.[1] The yellow and orange color seen on dermoscopy of JXG reflects the lipid-laden histiocytes and granulomatous inflammation present under the microscope, respectively.[2] Dermoscopic appearance of central orange–yellow background with surrounding erythematous border seen in JXG, has been given a metaphorical term “setting-sun pattern.”[3] Dense, diffuse histiocytic infiltrate present in dermis pushes the dermal vessels upward closer to skin surface which appear as well-focused linear and branching vessels on dermoscopy. The appearance of setting-sun pattern has also been described in dermoscopy of reticulohistiocytoma, Erdheim–Chester disease, and xanthoma disseminatum.[2] Clinical and dermoscopic findings of JXG and its differentials are discussed in Table 1. Considering the self-limited nature of JXG, it is of utmost importance to correctly diagnose JXG to avoid invasive procedures in children. Dermoscopy can act as an adjunctive tool to clinical examination in reaching a final diagnosis of JXG.[4]
Table 1.
Clinical and dermatoscopic findings of JXG and its differentials
| JXG and its differentials | Clinical presentation | Dermatoscopic findings |
|---|---|---|
| Juvenile xanthogranuloma | Asymptomatic, yellow, red, or brown, firm, rubbery, papulonodular lesions in infancy and childhood. | Orange-yellow background with a subtle erythematous border (setting sun appearance), clouds of paler yellow globules, linear and branched vessels. Whitish streak and subtle pigment network are other non-specific findings. |
| Pilomatricoma | Solitary, deep nodule with firm to stone-hard consistency at any age with female predominance. | Multiple, irregularly shaped, whitish structures and streaks with vascular structures in majority of cases. Ulceration and structureless gray-blue areas are other findings. |
| Epidermal cyst | Dermal or subcutaneous, slow growing nodules with central punctum usually in adults. | White, yellow, brown or black pore representing plugged pilosebaceous unit (pore sign). Bluish center with branching vessels in unruptured cyst; peripheral erythema with ivory-white color and linear vessels are seen in ruptured cyst. |
| Trichilemmal cyst (TC)[4] | Firm, commonly multiple nodules mainly over scalp. Seen more frequently in young females. | Not well described. Central yellowish-white patch with peripheral pigment network reported in TC over leg. One report described homogenous blue pigmentation with minimal reddish hue in TC over arm. |
| Present case of JXG | Asymptomatic, single, firm, reddish-orange nodule of approximately 1 cm × 1 cm | Diffuse orangish-yellow area surrounded by erythematous zone. Well-focused polymorphous vessels namely linear-irregular, looped, and branching vessels at center as well as periphery were also present. |
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The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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References
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