PMC3997242_01
(A) Slightly elevated erythematous papules covered by yellowish scales in the vertex of the scalp. (B) Reddish scaly papules at the temporal region. (C) Three ulcers at the perineum, one with elevation of the borders. [Copyright:  2012 Jeunon et al.]
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A 17-year-old white male presented to dermatological consultation with some asymptomatic, slightly elevated, erythematous papules covered by yellowish scales in the scalp vertex and temporal regions of two years' duration and small indurated ulcers with elevated borders at the perineum for six months (Figure 1).
(A) Slight bilateral gynecomastia and absence of terminal hairs in beard area and axillae. (B) Underdevelopment of genitalia in relation to age. [Copyright:  2012 Jeunon et al.]
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In addition to the skin lesions, physical exam showed slight bilateral gynecomastia, absence of terminal hairs in the beard area, axillae and pubis, as well as underdevelopment of testicles and penis (Figure 2).
(A) Langerhans cell histiocytosis, perineal lesion, scanning magnification. Note the dense band-like cellular infiltrate in the dermis and ulceration at the right side of the section (H&E, x20). (B) Band-like cellular infiltrate in the superficial and middle dermis. The intense pink areas correspond to clusters of eosinophils and the paler pink areas correspond to groups of abnormal Langerhans cells (H&E, x100). (C) Abnormal Langerhans cells with vesicular cleaved nucleus and numerous intermingling eosinophils (H&E, x400). (D) Cytological details of abnormal Langerhans cells. Note the amphophilic abundant cytoplasm and the large cleaved vesicular nucleus. The cell in the center of the photomicrograph has the appearance of a kidney (H&E, x1000). (E) Dense infiltrate of Langerhans cells forming a sheet in the dermis and blurring the dermoepidermal junction (H&E, x100X). (F) A sheet of abnormal Langerhans cells (H&E, x400). (G) Cytological details of abnormal Langerhans cells. Note the coffee bean and kidney shapes of the large cleaved nucleus (H&E, x1000). [Copyright:  2012 Jeunon et al.]
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In other foci, in which neoplastic cells were sparser, numerous intermingling eosinophils were present (Figure 3).