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Histopathological criteria of Degos`Disease (DD)

Histopathological criteria of Degos`Disease (DD). Matthias Steinhoff, MD, Christos C. Zouboulis, M.D., Ph.D. „Classical“ histological finding in DD. Robert Degos 1942: ... thrombosis of cutaneous vessels in the deep dermis resulting in a wedge-shaped area of dermal necrosis.

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Histopathological criteria of Degos`Disease (DD)

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  1. Histopathological criteria of Degos`Disease (DD) Matthias Steinhoff, MD, Christos C. Zouboulis, M.D., Ph.D.

  2. „Classical“ histological finding in DD Robert Degos 1942: ... thrombosis of cutaneous vessels in the deep dermis resulting in a wedge-shaped area of dermal necrosis ...

  3. Variable histological findings in DD „wedge-shaped zone of infarction“ 3 out of 27 biopsies (9 patients) „blood vessel thrombosis“ 2 out of 27 biopsies (9 patients) Su et al. Cutis 1985

  4. Additional histological features of DD: ·compact hyperkeratosis and atrophy of the epidermis · necrotic basal keratinocytes ·interface changes; vacuolar alteration ·perivascular /periadnexal lymphocytic infiltrates ·peri-/intraneural infiltrates ·lymphocytic vasculitis ·dermal mucin deposition Winkelmann et al. Arch Dermatol 1963; Strole et al. New Eng J Med 1967; Roenigk et al JAMA 1968; Feuerman et al Arch Path 1970; Soter et al. J AM Acad Dermatol 1982; Su et al. Cutis 1985; Grilli et al. Am J Dermatopathol 1999

  5. Robert Degos 1942: „... The cutaneous elements are of different ages and appear in crops...“

  6. Early fully developed late Harvell et al. Am J Dermatopathol 2001

  7. Histopathology of early lesions superficial and deep perivascular and periadnexal lymphocytic cell infiltrate Harvell et al. Am J Dermatopathol 2001

  8. Histopathology of early lesions interstitial mucin deposition Harvell et al. Am J Dermatopathol 2001

  9. Histopathology of early lesions subtle interface dermatitis; perineural and intraneural lymphocytic inflammation Harvell et al. Am J Dermatopathol 2001; Soter et al. J Am Acad Dermatol 1982

  10. Histopathology of fully developed lesions

  11. Histopathology of fully developed lesions

  12. Histopathology of fully developed lesions

  13. Histopathology of fully developed lesions

  14. Histopathology of fully developed lesions

  15. Histopathology of late lesions wedge-shaped area of dermal sclerosis Harvell et al. Am J Dermatopathol 2001

  16. Histopathology of late lesions in the wedge-shaped area of sclerosis the mucin is markedly diminished Harvell et al. Am J Dermatopathol 2001

  17. Histopathology of late lesions

  18. Summary • ·Not always “classical” histology in DD • · Histology varies in parallel to clinical evolution of the • individual papule • Pronounced lymphocytic infiltrate in early > late lesions • True lymphocytic vasculitis in fully developed (and late) lesions • Mucin in all stages ·Histological DD of DD: lupus erythematodes, dermatomyositis lichen sclerosus et athrophicans • · No specific, but highly sensitive histological features

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