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Classification of histiocytosis

Classification of histiocytosis. Class I histiocytosis : Langerhans cell histiocytosis Class I I a histiocytosis : Histiocytosis involving cells of the dermal dendrocyte lineage:

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Classification of histiocytosis

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  1. Classification of histiocytosis • Class I histiocytosis: Langerhans cell histiocytosis • ClassI I ahistiocytosis: Histiocytosis involving cells of the dermal dendrocyte lineage: • @ Dermatofibroma @ Juvenile xanthogranuloma @ Multicentric reticulohistiocytosis @ Benign cephalic histiocytosis @ Erdheim-Chester disease @ Fat- storing hamartoma of dermal dendrocytes @ Generalized eruptive histiocytoma @ Papular xanthoma @ Progressive nodular histiocytosis @ Xanthoma disseminatum • Class II b histiocytosis: Histiocytosis involving cells other than Langerhans cells& dermal dendrocytes: • @ Diffuse plane xanthoma @ Familial hemophagocytic lymphohistiocytocytosis @ Familial progressive mucinosis histiocytosis @ Malakoplakia @ Necrobiotic xanthogranuloma • Class III histiocytosis: malignant histiocytosis: • @ Monocytic leukemia @ malignant histiocytosis @ True histiocytic lymphoma

  2. Langerhans cell histiocytosisSYN: Histiocytosis x; eosinophilic granuloma; Letter- siwe disease; Hand-Schuller-Christian syndromeCLASS I histiocytosisCase 1

  3. The condition started as itchy papules on the face, then became more generalized. Many topical applications were used without any improvement.

  4. CD 1a

  5. CD 1a

  6. Langerhans cell histiocytosis • A group of disorders that have a broad spectrum of severity. • The course is variable with disease ranging from localized self limiting form to generalized fatal cases.

  7. Treatment • For mild single-system skin disease topical corticosteroids, topical antibacterial agents, PUVA, and topical nitrogen mustard have been reported to be effective.

  8. In multisystem LCH treatment should be initially with prednisolone 2 mg/kg for a short course of about 2 months. • Other agents have been tried as vinblastine, methotrexate, 6-mercaptopurine, etoposide & thalidomide.

  9. Progressive nodular histiocytosisCLASSIIa histiocytosis

  10. A 21 years old male complaining of multiple asymptomatic skin lesions over the extremities of 1 year duration of gradual onset & progressive course.

  11. S 100

  12. CD1a

  13. CD45 Ro

  14. CD20

  15. CD 68

  16. Progressive nodular histiocytosis • A histiocytic disorder with a progressive deforming course. • The eruption consists of two different types of lesions, superficial papules and deep nodules • Despite the progressive nature of the disease, patients remain in good health.

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