A case of malignant atrophic papulosis with cranial nerve and peripheral nerve impairment

Liu, Fang; Li, Haibo; Zhang, Min; Yan, Wenliang; Sang, Hong

doi:10.1590/abd1806-4841.20153490

Cited by 3 publications

(1 citation statement)

References 9 publications

(10 reference statements)

Supporting

Mentioning

Contrasting

Order By: Relevance

“…There is an increased level of proteins in the cerebrospinal fluid [58,60]. Cranial and peripheral nerves can be affected [54,57,58,[61][62][63].…”

Section: Nervous System Involvementmentioning

confidence: 99%

Atrophic Papulosis

Robert¹,

Hot²

2022

Dermatology

View full text Add to dashboard Cite

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.

show abstract

“…There is an increased level of proteins in the cerebrospinal fluid [58,60]. Cranial and peripheral nerves can be affected [54,57,58,[61][62][63].…”

Section: Nervous System Involvementmentioning

confidence: 99%

Atrophic Papulosis

Robert¹,

Hot²

2022

Dermatology

View full text Add to dashboard Cite

show abstract

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

Kim

Kashetsky

Bagit

et al. 2021

Acad Dermatol Venereol

View full text Add to dashboard Cite

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.

show abstract

Malignant atrophic papulosis treated with eculizumab and hirudin: a fatal case report and literature review

Yu,

Wang,

Tang

et al. 2024

Front. Cardiovasc. Med.

View full text Add to dashboard Cite

BackgroundMalignant atrophic papulosis (MAP) is a rare obliterative vasculopathy whose etiology and pathophysiological mechanisms remain unknown, and the treatment is still empirical. It can involve multiple systems, especially the gastrointestinal tract and central nervous system, and has a poor prognosis.Case presentationA 20-year-old Chinese male appeared to have Widespread atrophic papules and plaques, intermittent abdominal pain, recurrent bowel perforation, and psoas abscess. The clinical diagnosis of MAP was supported by skin biopsy. He was then treated with anticoagulants, antiplatelets, glucocorticoids, and immunosuppressants and started on eculizumab and hirudin after the first surgical interventions. Despite the aggressive immunosuppression, anticoagulant, antiplatelet, humanized monoclonal antibodies, and surgery therapy, he died five months after presentation.ConclusionsMAP is an extremely rare obliterative vasculopathy manifesting as benign cutaneous involvement or potentially malignant systemic involvement. MAP patients who exhibit any abdominal symptoms should undergo laparoscopy and evaluation in time and start on eculizumab and treprostinil as soon as possible, as the combination of them is presently the most effective treatment option for gastrointestinal MAP and hopefully reduce mortality.

show abstract

A case of malignant atrophic papulosis with cranial nerve and peripheral nerve impairment

Cited by 3 publications

References 9 publications

Atrophic Papulosis

Atrophic Papulosis

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

Malignant atrophic papulosis treated with eculizumab and hirudin: a fatal case report and literature review

Contact Info

Product

Resources

About