case_images: PMC9365013_01
case_images: Comparison of pre-treatment and post-treatment features in Case 1. Tan-Orange papules and plaques on the face (A,B,C) before treatment. The patient was treated with topical 1% rapamycin ointment twice daily for 24 weeks. Most of the skin lesions on the face flattened and faded (D,E,F).
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case_images: Dermatological examination revealed multiple lesions in the form of tan-orange papules and plaques on the face, ears (Figure 1A-C), trunk, both upper arms, and legs. After 24 weeks of using topical rapamycin on both sides, most of the skin lesions on his face had flattened and faded (Figure 1D-F).
case_images: Supporting examination of Case 1. (A) Dermoscopic examination revealed a brownish-yellow hue resembling a "setting-sun appearance". (B) Histopathological features of the skin lesions: (1) Infiltration of lymphocytes, foam cells, and histiocytes in the upper dermis. (2) Histiocytes forming Touton giant cells (red circle). (C) Immunohistochemistry analysis: (1) CD68 positive. (2) S100 positive in Langerhans cell and nerve cell. (3) CD1a negative.
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case_images: Dermoscopy examination showed a brownish-yellow hue resembling "setting-sun appearance" (Figure 2A). Additionally, histiocytes-forming Touton giant cells were also observed, supporting the diagnosis of JXG (Figure 2B). Furthermore, the immunohistochemistry analysis revealed that the cells were positive for CD68 and S100 (Langerhans cell and nerve cell), and negative for CD1a (Figure 2C).
case_images: PMC9365013_02
case_images: Comparison of pre-treatment and post-treatment features in Case 2. Multiple tan-Orange nodules on the scalp (A-C) before treatment. The patient was treated with topical 1% rapamycin cream twice daily for 24 weeks. Most of the skin lesions on the scalp decrease in size and faded (D-F).
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case_images: On physical examination, the lesions presented as multiple, discrete, asymptomatic, tan-orange nodules on the scalp and papules on the retroauricular region, face, and chest, with variable sizes, with the largest one being 15 mm in diameter (Figure 3A-C). Treatment was continued until 24 weeks for the rest of the BCH lesions on the scalp, where most of the skin lesions decreased in size and faded (Figure 3D-F).
case_images: Supporting examination of Case 2. (A) Dermoscopic examination revealed brownish-yellow structureless center with a pale erythematous halo lesion was observed, indicating a "setting-sun appearance". (B) Histopathological features of the skin lesions fibrocollagenous connective tissue on the dermis with massive histiocyte infiltration. (C) Immunohistochemistry analysis: (1) CD68 positive, (2) S100 weak positive for S100. (3) CD1a negative.
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case_images: Upon dermoscopy examination, a brownish-yellow structureless center with a pale erythematous halo lesion was observed, indicating a "setting-sun appearance" (Figure 4A). Histopathological examination revealed fibrocollagenous connective tissue on the dermis with massive histiocytic infiltration (Figure 4B). On immunohistochemistry analysis, the cells stained positive for CD68, but weak positive for S100 and negative for CD1a (Figure 4C).