Syn .: Progressive nodular histiocytoma, progressive nodular histiocytosis, spindle cell xanthogranuloma, progressive nodular histiocytoma, lipomatous histiocytoma.
Def .: A rare form of histiocytosis characterized by the spreading of two different types of lesions: multiple superficial papules or deep nodules.
Etiol .: No etiological factors.
Loc .: Accidental with a predilection to flexion surfaces. The changes may occur in the oral cavity, larynx and conjunctival mucosa.
Clinical: Yellow-orange papules 2 to 10 mm in size, skin-colored or reddish orange nodules 1 to 5 cm in size, with up to 100 or more lesions. Larger tumors are covered with skin with lots of telangiectasia, they can be painful, and occasionally bleed or necrotize. Over the years, new lesions develop, although the patients are in good general condition, the quality of life is impaired due to disfiguring eruptions, especially on the face (the so-called lion’s face). Internal organs are not involved.
Hist .: The lesions are located in the dermis without epidermotropism and the involvement of the epidermis. Early lesions show an accumulation of shell-like histiocytes, foam cells, and giant cells with slight lymphocyte infiltration. In older lesions, the histiocytes are fusiform. Giant cells may exceptionally be present. Cells are immunocytochemically positive for CD68 and factor XIIIa and negative for S100 and CD1a.
But: There is no effective treatment.
Lit.: [1] Gibbs N.F., O’Grady T.C.: Progresive nodular histiocytomas. J Am Acad Dermatol 1996, 35; 323-5.
Source: A. Kaszuba, Z. Adamski: “Lexicon of dermatology”; XNUMXst edition, Czelej Publishing House