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Histopathological criteria of DegosDisease (DD)
Matthias Steinhoff, MD, Christos C. Zouboulis,
M.D., Ph.D.
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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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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
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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
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Robert Degos 1942 ... The cutaneous elements
are of different ages and appear in crops...
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Early fully developed
late
Harvell et al. Am J Dermatopathol 2001
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Histopathology of early lesions
superficial and deep perivascular and
periadnexal lymphocytic cell infiltrate
Harvell et al. Am J Dermatopathol 2001
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Histopathology of early lesions
interstitial mucin deposition
Harvell et al. Am J Dermatopathol 2001
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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
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Histopathology of fully developed lesions
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Histopathology of fully developed lesions
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Histopathology of fully developed lesions
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Histopathology of fully developed lesions
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Histopathology of fully developed lesions
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Histopathology of late lesions
wedge-shaped area of dermal sclerosis
Harvell et al. Am J Dermatopathol 2001
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Histopathology of late lesions
in the wedge-shaped area of sclerosis the mucin
is markedly diminished
Harvell et al. Am J Dermatopathol 2001
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Histopathology of late lesions
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Summary

• Not always classical histology in DD
• Histology varies in parallel to clinical
  evolution of the
• individual papule
• Pronounced lymphocytic infiltrate in early gt
  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