DermatopathologyIs Degos' disease a clinical and histological end point rather than a specific disease?☆
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)
- et al.
Malignant atrophic papulosis (Degos' disease) involving three generations of a family
J Am Acad Dermatol
(1997) - et al.
Lymphocytes and necrosis of the cutaneous microvasculature in malignant atrophic papulosisa refined light microscope study
JAm Acad Dermatol
(1982) - et al.
The pathology and pathogenesis of malignant atrophic papulosis (Degos' disease)a case study with reference to other vascular disorders
Pathol Res Pract
(1987) - et al.
Degos' disease in a patient with acquired immunodeficiency syndrome
J Am Acad Dermatol
(1998) - et al.
Degos' diseasea 10 year follow-up of a patient without visceral involvement
J Am Acad Dermatol
(1989) - et al.
Thrombotic accidents induced by thalidomidetwo cases
Rev Med Interne
(2002) - Kohlmeier W. Multiple hautnekrosen bei thromboangitis obliterans. Arch Dermatol Syphilol...
- et al.
Dermatite papulo-squamese atrophiante
Bull Soc Fr Dermatol Syphiligr
(1942) - et al.
Benign cutaneous Degos' disease
Br J Dermatol
(1998) - et al.
Benign cutaneous Degos' disease. A case report with emphasis on histopathology as papules chronologically evolve
Am J Dermatopathol
(2001)
Familial malignant atrophic papulosis (Degos' disease) additional evidence for heredity and a benign course
Br J Dermatol
Atrophie blanche lesions closely resembling malignant atrophic papulosis (Degos' disease) in systemic lupus erythematosus
Br J Dermatol
Skin lesions resembling malignant atrophic papulosis in lupus erythematosus
Dermatologica
Systemic lupus erythematosus resembling malignant atrophic papulosis
Arch Int Med
Progressive systemic sclerosis with malignant atrophic papulosis
Arch Dermatol
Lesions resembling malignant atrophic papulosis in a patient with dermatomyositis
J Am Acad Dermatol
Degos' diseaseassociation with anticardiolipin antibodies and the lupus anticoagulant
Br Med J
Cited by (66)
Skin
2021, Lahita’s Systemic Lupus ErythematosusJuvenile dermatomyositis: A series of 22 cases
2020, Annales de Dermatologie et de VenereologieDiagnostic Pathology: Nonneoplastic Dermatopathology
2016, Diagnostic Pathology: Nonneoplastic DermatopathologyCutaneous manifestations of gastrointestinal disease: Part II
2013, Journal of the American Academy of DermatologyCitation Excerpt :Given the distinct presentations of BAP and MAP along with the incompletely understood pathophysiology, DD might better be considered a syndrome at this juncture. There are those, in fact, who believe that DD might be the end result of vascular insult rather than its own distinct clinical entity.187 Common to both BAP and MAP are skin lesions.
Skin
2011, Systemic Lupus ErythematosusClinical Associations of Degos-Like Lesions in Patients with Systemic Sclerosis
2023, JAMA Dermatology
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Funding sources: None.
Conflicts of interests: None identified.