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 DD

Su 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 late

Harvell et al. Am J Dermatopathol 2001

Histopathology of early lesions

Harvell et al. Am J Dermatopathol 2001

superficial and deep perivascular and periadnexal lymphocytic cell infiltrate

Histopathology of early lesions

Harvell et al. Am J Dermatopathol 2001

interstitial mucin deposition

Histopathology of early lesions

Harvell et al. Am J Dermatopathol 2001; Soter et al. J Am Acad Dermatol 1982

subtle 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 lesions

Harvell et al. Am J Dermatopathol 2001

wedge-shaped area of dermal sclerosis

Histopathology of late lesions

Harvell et al. Am J Dermatopathol 2001

in 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