A fatal case of malignant atrophic papulosis in a pediatric patient

Schaefer, L.; Muskardin, Theresa Wampler; Tillema, Jan‐Mendelt; Wieland, C; Tollefson, Megha M.

doi:10.1111/pde.14878

Cited by 5 publications

(7 citation statements)

References 17 publications

(29 reference statements)

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“…Treprostinil was not used in the patient due to high costs and insurance challenges. However, although eculizumab and treprostinil have been continued, progressive neurologic, pleural, and pericardial MAP has been reported ( 4 , 35 ). A recent study has revealed that the obliterative proliferative neointimal arteriopathy and the sclerodermic fibrosing serositis occur independently of complement inhibition, and both of them occurred in patients exhibiting complete blockade of complement on eculizumab ( 4 ).…”

Section: Discussionmentioning

confidence: 99%

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

Yu,

Wang,

Tang

et al. 2024

Front. Cardiovasc. Med.

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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.

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Section: Discussionmentioning

confidence: 99%

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

Yu,

Wang,

Tang

et al. 2024

Front. Cardiovasc. Med.

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“…The available data on pAP mainly derives from case reports and, interestingly, from articles mostly published in neurological journals (Table 1). 3,6,9–41 Atrophic papulosis is considered to affect middle‐aged adults. However, a substantial number of patients in our cross–sectional study were children (20%; 19/96).…”

Section: Discussionmentioning

confidence: 99%

“…However, the number of case reports of paediatric atrophic papulosis (pAP) is constantly growing (Table 1). 3,6,9–41 Our study aimed to investigate the early occurrence of the disease, including congenital manifestations, and to compare this to adult atrophic papulosis (aAP).…”

Section: Introductionmentioning

confidence: 99%

Atrophic papulosis (Köhlmeier–Degos disease) in children and adolescents—A cross–sectional study and literature review

Zouboulis

Kaleta

Broniatowska

et al. 2023

Acad Dermatol Venereol

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Background: Atrophic papulosis (Köhlmeier-Degos disease, Degos disease) is a rare thrombo-obliterative microangiopathy of unknown pathogenesis. It usually affects people between the ages of 20 and 50. However, it can occur at any age. The condition is considered uncommon in children. Objective: Clinical characterization of paediatric patients with atrophic papulosis. Methods: Single-centre prospective cohort study with data derived from the international Degos Disease Registry collected between 2000 and 2021.Results: Among 96 registered patients with atrophic papulosis fulfilling the criteria, 19 were aged 0 to completed 17 years at the time of onset. The median age at the time of onset was 5 years, ranging from 0 to 1 years for girls to 8 years for boys. In contrast to adult patients (male-to-female ratio 1:2.2), there was a male predominance in paediatric patients with a male-to-female ratio of 1.7:1. Systemic involvement, in particular gastrointestinal, central nervous system and cardiac, was more frequent in children than in adult patients. There were no statistically significant differences between family history, multisystem involvement, mortality and median survival time in the two groups. Conclusions: Atrophic papulosis has some distinct features in the paediatric population. It presents an important and still under-recognized problem. Therefore, it is mandatory to pay attention to the typical skin lesions in combination with neurological or gastrointestinal symptoms in order to make a prompt and accurate diagnosis.How to cite this article: Zouboulis CC, Kaleta KP, Broniatowska E, Jarienė V, Nikolakis G. Atrophic papulosis (Köhlmeier-Degos disease) in children and adolescents-A cross-sectional study and literature review.

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“…A case of cutaneous necrotizing eosinophilic vasculitis was successfully treated with prednisolone, pentoxifylline, and nifedipine 15 . Three cases describe the use of pentoxifylline in conjunction with other therapies for malignant atrophic papulosis 16–18 …”

Section: Vascularmentioning

confidence: 99%

“…15 Three cases describe the use of pentoxifylline in conjunction with other therapies for malignant atrophic papulosis. [16][17][18]…”

Section: Vasculitis and Vasculopathiesmentioning

confidence: 99%

Pentoxifylline in dermatology

Balazic

Axler

Konisky

et al. 2022

J of Cosmetic Dermatology

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Pentoxifylline was initially marketed for use in patients with intermittent claudication due to chronic occlusive arterial disease of the extremities but has since been shown to have several off-label uses. It can be described chemically as 1-(5-oxohexyl)-3, 7-dimethylxanthine, which is a tri-substituted xanthine derivative. 1 Pentoxifylline has a variety of mechanisms of action (Figure 1). In its original purpose, it was used because of its rheological modifying properties, such as increasing the deformability of erythrocytes

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A fatal case of malignant atrophic papulosis in a pediatric patient

Cited by 5 publications

References 17 publications

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

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

Atrophic papulosis (Köhlmeier–Degos disease) in children and adolescents—A cross–sectional study and literature review

Pentoxifylline in dermatology

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