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Histopathological criteria of Degos`Disease (DD)

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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. - PowerPoint PPT Presentation

Text of Histopathological criteria of Degos`Disease (DD)

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

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

  • wedge-shaped zone of infarction

    3 out of 27 biopsies (9 patients)

    blood vessel thrombosis

    2 out of 27 biopsies (9 patients)Variable histological findings in DDSu et al. Cutis 1985

  • 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

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

  • Early fully developed lateHarvell et al. Am J Dermatopathol 2001

  • Histopathology of early lesionsHarvell et al. Am J Dermatopathol 2001superficial and deep perivascular and periadnexal lymphocytic cell infiltrate

  • Histopathology of early lesionsHarvell et al. Am J Dermatopathol 2001interstitial mucin deposition

  • Histopathology of early lesionsHarvell et al. Am J Dermatopathol 2001; Soter et al. J Am Acad Dermatol 1982subtle interface dermatitis;

    perineural and intraneural lymphocytic inflammation

  • Histopathology of fully developed lesions

  • Histopathology of fully developed lesions

  • Histopathology of fully developed lesions

  • Histopathology of fully developed lesions

  • Histopathology of fully developed lesions

  • Histopathology of late lesionsHarvell et al. Am J Dermatopathol 2001wedge-shaped area of dermal sclerosis

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

  • Histopathology of late lesions

  • 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