Keywords

Case Presentation

Male patient 2 years old presented in the dermatologic clinic with single asymptomatic nodule on his left buttock (Fig. 17.1). The lesion had history of 6 months; the patient mother told he had no relevant familial or personal medical history. On physical examination of the skin, he had single asymptomatic yellow-brown, well-defined, soft to stiff, pedunculated papulo-nodular lesion with size 2 × 2 cm and some subcutaneous patterns. The examination of mucous membranes, palms, other areas of the skin, eyes and soles revealed normal findings. There was no evidence of apparent inguinal lymph node enlargement. Initially the patient tried topical steroids and antibiotic with no response.

Fig. 17.1
figure 1

Single asymptomatic nodule on the left buttock of male patient 2 years old

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Dermoscopic examination revealed a red-yellow center and a discrete erythematous halo, characterizing the “setting sun” pattern (Fig. 17.2).

Fig. 17.2
figure 2

Dermoscopic examination revealed “setting sun” pattern: a red-yellow center and a discrete erythematous halo (Dermoscopy 3gen DermLite 3, magnification 10×)

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Based on the Case Description, Clinical and Dermoscopic Photographs, What Is Your Diagnosis?

  1. 1.

    Hemangioma.

  2. 2.

    Xanthogranuloma.

  3. 3.

    Gluteal abscess.

  4. 4.

    Rosai-Dorfman disease

Diagnosis

Juvenile xanthogranuloma

Discussion

Juvenile Xanthogranuloma (JXG) is a non-Langerhans cell histiocytosis disorder [1]. The lesions usually present as asymptomatic yellow-brown papules and nodules that are mostly self-limited. JXG can present anywhere in the body but primarily occurs as a solitary lesion on the head, the neck, or upper trunk [1].

Skin lesions of JXG are categorized into the three main forms of small nodular/popular (2–5 mm), large nodular (5–20 mm), and giant xanthogranuloma (>20 mm) [2]. In most juvenile cases, lesions regress markedly by a year [3].

Dermoscopy of juvenile Xanthogranuloma revealed characteristic pattern, the pattern described as setting sun is characterized by a yellow-orange central area, which may show areas of lighter yellow, correlating with the dermal xanthogranulomatous infiltrate, and an erythematous halo, which may occur at any stage of evolution [4]. Linear telangiectasias have also been described. Other non-specific characteristics found include discrete pigment network, whitish streaks indicating areas of fibrosis, and fine, branched vessels [4].

Key Points

  • Juvenile Xanthogranuloma (JXG) is a relatively uncommon non-Langerhans cell histiocytosis disorder.

  • Dermoscopy can help in diagnosis and differnate it from other diseases by characteristic sunset pattern.