Dermatopathology
Is Degos' disease a clinical and histological end point rather than a specific disease?

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Abstract

Degos' disease is described as a rare disorder, with approximately 100 cases detailed in the literature. Nearly all are characterized by the near “pathognomonic” appearance of porcelain-white, atrophic papules with peripheral erythema and telangiectases. Many Degos' disease variants have been described including benign cutaneous Degos' disease, familial Degos' disease, atrophie blanche with Degos'-like features, and connective tissue diseases with similar findings. The course, prognosis, and treatment have substantially varied. We present four patients: the first carries a diagnosis compatible with classic Degos' disease, the second and third demonstrate cutaneous and histological findings of Degos' disease but laboratory evidence suggestive of lupus erythematosus, while the fourth has dermatomyositis with Degos'-like lesions. Because of broad overlap in clinical and histological findings, we contend that Degos' disease may not be a specific entity, but rather, may represent a common end point to a variety of vascular insults, many of which have not been fully elucidated.

Section snippets

Case 1

A 56-year-old woman was referred for a papular eruption of 6 years duration. Lesions would progress from fleshy papules to atrophic scars with rosy erythema and telangiectases (Fig 1). They began on the extremities and progressed to involve the trunk.

Prednisone and hydroxychloroquine had been ineffective. Past medical history was significant for a perforated gastric ulcer. Family history was noncontributory. Anti-nuclear antibody (ANA), extractable nuclear antigen (ENA), complement, and

Discussion

Degos' disease, also known as malignant atrophic papulosis, is an uncommon condition with reportedly stereotypical skin lesions consisting of largely asymptomatic, porcelain-white, atrophic papules, with surrounding erythema and telangiectases.13, 14, 15 While the etiology is unknown, evidence suggests a vaso-occlusive process. It has been alternatively regarded as an obliterating arteriolitis, necrotizing vasculitis, endovasculitis with secondary thrombosis, intravascular coagulation disorder,

Conclusion

In summary, we propose that Degos' disease is, perhaps, not a distinct clinical entity, but rather that it represents a common clinical and histological endpoint to vascular insult. Considerable blurring and incomplete characterization of the original defining cases of malignant atrophic papulosis exist. It is likely that many historical cases were associated with antiphospholipid antibody and/or lupus erythematosus; however, confirmatory testing was not then available. We assert that several

References (36)

  • L Habbema et al.

    Familial malignant atrophic papulosis (Degos' disease) additional evidence for heredity and a benign course

    Br J Dermatol

    (1986)
  • M.M Black et al.

    Atrophie blanche lesions closely resembling malignant atrophic papulosis (Degos' disease) in systemic lupus erythematosus

    Br J Dermatol

    (1976)
  • M.S Doutre et al.

    Skin lesions resembling malignant atrophic papulosis in lupus erythematosus

    Dermatologica

    (1987)
  • H.V Dubin et al.

    Systemic lupus erythematosus resembling malignant atrophic papulosis

    Arch Int Med

    (1974)
  • B.G.M Durie et al.

    Progressive systemic sclerosis with malignant atrophic papulosis

    Arch Dermatol

    (1969)
  • H Tsao et al.

    Lesions resembling malignant atrophic papulosis in a patient with dermatomyositis

    J Am Acad Dermatol

    (1997)
  • H.J Englert et al.

    Degos' diseaseassociation with anticardiolipin antibodies and the lupus anticoagulant

    Br Med J

    (1984)
  • Degos R, Kalis B. La papulose atrophiante maligne. Ann Med Interne...
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    Funding sources: None.

    Conflicts of interests: None identified.

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