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Histiositosis ditandai oleh adanya infiltrasi dan akumulasi histiosit dalam jaringan. Penyakit ini jarang ditemukan dan etiologinya belum diketahui secara pasti.Dahulu diduga merupakan suatu proses neoplastik.  Saat ini diduga histiositosis merupakan suatu proses reaktif. Kelainan ini dapat bersifat akut maupun kronik dengan manifestasi klinis yang bervariasi, dari ringan sampai mengancam hidup. Kelainan kulit adalah salah satu manifestasi klinis yang sering ditemukan. Terdapat 3 kelompok histiositosis, yaitu histiositosis sel Langerhans, histiositosis non sel Langerhans, dan proliferasi klonal maligna. Sampai saat ini belum ada terapi yang bersifat definitif. Terapi yang diberikan bertujuan untuk mencegah kerusakan jaringan yang ireversibel. Prognosis penyakit ini sulit diperkirakan, namun dipengaruhi oleh usia saat didiagnosis, jumlah organ terlibat dan disfungsi organ saat didiagnosis, serta respons penyakit terhadap pengobatan. Terapi bertujuan mencergah kerusakan menetap jaringan normal oleh lesi histiositosis. Dengan penatalaksanaan yang tetap diharapkan pasien mempunyai harapan untuk mengalami remisi dan kesembuhan total. Diperlukan penelitian lebih lanjut untuk memahami pathogenesis hingga terapi yang sesuai yang dapat diberikan. (MDVI 2007;34/1:86-98)

Kata kunci: histiositosis, kelainan kulit

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Histiocytosis is characterized by infiltration and accumulation of hystiocytes in body tissues. This disease is rare with unknown etiology. Currently the theory of reactive process is being sought. It can be acute or chronic in nature. Clinical manifestation varies from mild to life threatening. Cutaneous involvement is one of the clinical manifestations which is often found. Histiocytosis is divided into 3 groups; Langerhans cell histiocytosis, non-Langerhans cell histiosytosis, and malignant clonal proliferation. Definitive therapy for histiocytosis has not yet established. The aim of treatment is to prevent irreversible tissue destruction. The prognosis is unpredictable, under influenced of age of patient, number of affected organs, presence of organ dysfunction when diagnosis is made, and response to treatment. With good treatment, patient have new hope for remission dan total healing. Continous studies must be done to know the pathogenesis untilthe new treatment for the disease. (MDVI 2007;34/1:86-98)

Keywords: Histiositosis, cutaneous involvement