Benign form of atrophic papulosis developed at injection sites of pegylated-alpha-interferon: is there a pathophysiological link?

Zaharia, Danièla; Truchot, F.; Ronger-Savlé, S.; Balme, B.; Thomas, Luc

doi:10.1111/bjd.12773

Cited by 4 publications

(5 citation statements)

References 10 publications

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“…Finally, there are theories of genetic predisposition, where first‐degree relatives are affected more often suggesting an autosomal dominant inheritance pattern. Genetic inheritance appears to be associated more commonly with the cutaneous Degos variant 38,87,90,117,123,161,180,226,252 . In our results, a notable family history was reported more often in BAP (21.8%, n = 27/124) compared with MAP (3.5%, n = 8/228).…”

Section: Discussionsupporting

confidence: 53%

“…Genetic inheritance appears to be associated more commonly with the cutaneous Degos variant. 38,87,90,117,123,161,180,226,252 In our results, a notable family history was reported more often in BAP (21.8%, n = 27/124) compared with MAP (3.5%, n = 8/ 228). Overall, the different mechanisms of Degos pathogenesis being studied to date involve coagulation disorders, autoimmunity, viral infections, 230 vasculitis or primary dysfunction of endothelial cells.…”

Section: Treatments and Outcomessupporting

confidence: 50%

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Clinical manifestations and treatment outcomes in degos disease: a systematic review

Kim

Kashetsky

Bagit

et al. 2021

Acad Dermatol Venereol

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Degos disease (atrophic papulosis) is a rare vasculopathy with cutaneous and systemic manifestations. Although potentially fatal, the characteristics of and treatments for Degos disease variants are not adequately described. We conducted a systematic review to summarize cutaneous and systemic presentations, treatments and outcomes of malignant (MAP) and benign (BAP) variants of Degos disease. A comprehensive search was conducted on Embase, MEDLINE, CINAHL and CENTRAL on 27 October 2020, which yielded 254 original studies reporting cases of Degos disease. A total of 357 patients were included in the analysis. Mean age of onset was 33.9 years. MAP was most commonly reported (63.8%, n = 228/357), with 56.6% (n = 129/228) mortality. Cutaneous lesions were usually asymptomatic (26.3%, n = 81/308) and localized to the trunk (57.7%, n = 206/357) and extremities (56.8%, n = 203/357). Systemic involvement developed within 2 years on average, ranging from 0 to 28 years. Anti-platelet monotherapy had a complete resolution rate of 42.3% (n = 11/26) in BAP and 20.0% (n = 7/35) in MAP. Based on the findings of the study, most cases of Degos disease are malignant with high mortality, and even benign cutaneous cases may develop systemic disease in as late as 28 years. Anti-platelet monotherapies may prove effective against both variants. Further studies are needed to confirm these findings.

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Section: Discussionsupporting

confidence: 53%

Section: Treatments and Outcomessupporting

confidence: 50%

Clinical manifestations and treatment outcomes in degos disease: a systematic review

Kim

Kashetsky

Bagit

et al. 2021

Acad Dermatol Venereol

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“…A patient with HIV infection presented cutaneous lesions of AP without evidence of visceral involvement. Similar findings are described in an HCV patient treated with pegylated alpha interferon (IFN) and ribavirin [25,26]. Almost 8% of patients have a history of HCV infection and 6% of herpes zoster [10].…”

Section: Genetic Predispositionsupporting

confidence: 61%

“…Moreover, antiphospholipid antibodies have been found in some cases, but the presence of lupus anticoagulant may be unspecific [18,[27][28][29][30][31]. Immune deficiencies and immunosuppressive and immunomodulatory treatments can impact the disease course [25,26,32].…”

Section: Genetic Predispositionmentioning

confidence: 99%

“…Type I IFN systemic levels are also increased in AP, as assessed by a high IFN score and an increase of CD169+ monocytes in the blood [16,24]. The role of type I IFN in AP pathogenesis is also suggested by the appearance of typical skin lesions at injection sites of pegylated-IFNα therapy and by the possibility of a viral infection as a trigger for the disease [26]. Overall, growing evidence suggests that there is an IFN-and complement-mediated lymphocytic microvascular injury that underlies AP vasculopathy [37].…”

Section: Vasculopathymentioning

confidence: 99%

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Atrophic Papulosis

Robert¹,

Hot²

2022

Dermatology

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Background: Atrophic papulosis (AP) is a rare obliterating vasculopathy characterized by specific skin lesions. The etiology and the pathophysiology of the disease remain unclear. The treatment is still empirical, while the malignant form of the disease is associated with a poor prognosis. Summary: The underlying pathogenesis of AP includes three mechanisms with vasculopathy, coagulopathy, and endothelial dysfunction. Benign and malignant forms of AP are described. The benign form is confined to the skin. The pathognomonic skin lesions evolve over time and are large papules with an atrophic porcelain-white center and an erythematous rim. However, systemic involvement can occur months or years after the initial skin features. In this latter case, the associated mortality is very high with a mortality rate of over 65% in some series. Gastrointestinal involvement and central nervous system infarctions are the most frequent causes of death. Treatment is empirical with the use of antiplatelet therapy, anticoagulants, steroids, intravenous immunoglobulins, and immunosuppressive agents. Recent evidence shows that eculizumab, a complement inhibitor, is the most effective therapy in malignant AP with gastrointestinal involvement of the disease and should be combined with treprostinil to prevent relapse.

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Peginterferon-α

2014

Reactions Weekly

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A man [age at reaction onset not clearly stated] developed atrophic papulosis during treatment with peginterferon-α.The man, who had a history of hepatitis type C and ulcerative colitis, presented at the age of 38 years with a 1-year history of slightly painful red papules at the site of peginterferon-α injections. The papules appeared a few weeks after starting therapy with peginterferon-α [dosage not stated] and ribavirin. Cutaneous examination showed more than fifty 3-5mm lesions located on the limbs and trunk. Most of the lesions comprised papules with a slightly depressed, porcelain-white atrophic centre, with a slightly raised reddish rim. Almost all of the lesions were found in the cutaneous injection sites of peginterferon-α. A biopsy specimen revealed an area of degenerated collagen with the apex extending into the dermis. Thrombotic and lymphocytic vasculitis was also detected. A diagnosis of atrophic papulosis was established.The man's peginterferon-α was discontinued and there was no relapse during a 3-year follow-up.Author comment: "[T]he precise locations of the cutaneous lesions at injection sites are in favour of a direct role of this agent".

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Benign form of atrophic papulosis developed at injection sites of pegylated-alpha-interferon: is there a pathophysiological link?

Cited by 4 publications

References 10 publications

Clinical manifestations and treatment outcomes in degos disease: a systematic review

Clinical manifestations and treatment outcomes in degos disease: a systematic review

Atrophic Papulosis

Peginterferon-α

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