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  • Langerhans cell histiocytosis: Translucent, rose-yellowish, crusted papules or papulovesicles, eczematous lesions, hemorrhagic papules and nodules, petechiae, noduloulcerative mucosal lesions, and nail involvement

  • Rosai-Dorfman disease: Lymphadenopathy, cutaneous nodules, and plaques

  • Hemophagocytic lymphohistiocytosis: Various cutaneous manifestations such as erythroderma, generalized purpuric macules and papules, and morbilliform eruptions

  • Juvenile xanthogranuloma: Solitary as well as multiple (oligolesional) papules or nodules—early lesions show a reddish-brown color; mature lesions have a reddish-yellow appearance

  • Benign cephalic histiocytosis: Like juvenile xanthogranuloma; ultrastructural presence of worm-like bodies

  • Generalized eruptive histiocytoma of childhood: Widespread, erythematous, essentially symmetrical papules

  • Adult xanthogranuloma: Oligolesional, yellow-orange papules that usually appear on the face, neck, and lower arms

  • Papular xanthoma: Solitary yellowish papule

  • Generalized eruptive histiocytoma: Multiple asymptomatic and symmetrically distributed brownish erythematous papules, particularly involving the axial regions such as the trunk, face, and proximal extremities, frequently flares

  • Xanthoma disseminatum: Small, yellow-red to brown papules and nodules that are discrete and are disseminated with a predilection for the flexural and intertriginous areas, as well as for mucous membranes

  • Multicentric reticulohistiocytosis: Firm, yellow-brownish papules or nodules that reach a size of several centimeters and progress slowly in size; lesions over the joints of fingers and wrists are typical; often involvement of mucosae and conjunctivae

  • Erdheim-Chester disease: Xanthelasma and xanthoma are present in one-sixth of cases; long bone and other extracutaneous involvement

  • Necrobiotic xanthogranuloma: Yellowish plaques and nodules that can ulcerate

  • Hereditary progressive mucinous histiocytosis: Skin-colored to red-brown papules that usually develop in the first decade on the nose, hands, forearms, and thighs that can later develop into persistent and progressive erythematous papules

  • Progressive nodular histiocytosis: Generalized, discrete yellow to red-brown papules and nodules measuring a few centimeters in size with prominent facial involvement

Langerhans cell histiocytoses (LCHs) and non–Langerhans cell histiocytoses (N-LCHs) present a rare group of neoplastic and reactive diseases characterized by the proliferation of myeloid cells in various organ sites with a predilection to the skin. As shown in Table 117-1, they are categorized in different subgroups.

Table 117-1Classification of Langerhans and Non–Langerhans Cell Histiocytosis



  • New definition of Langerhans cell histiocytosis (LCH) as rare, inflammatory neoplasia of myeloid-dendritic (mostly clonal) cells with heterogeneous clinical manifestation

  • Histopathology:

    • Infiltration of the organs with immature, morphologically rounded myeloid dendritic cells with bean-shaped nuclei; shared characteristics of ...

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