Disseminated Whitish Papules and Scars on a Patient With Bowel Perforation

Partarrieu-Mejías, Felipe; Llancapi, Patricio; Monardes, Jorge

doi:10.1001/jamadermatol.2017.2174

Cited by 2 publications

(1 citation statement)

References 10 publications

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“…Deaths from gastrointestinal K-D resulted from recurrent bowel perforation, with hemorrhage, hypotension, and sepsis. In our review, 59% (19/32) of those with gastrointestinal perforations died of sepsis or severe peritonitis [ 2 , 4 – 6 , 10 , 15 , 17 , 19 , 20 , 24 , 27 , 30 , 31 , 55 ].…”

Section: Treatmentmentioning

confidence: 99%

Gastrointestinal Kohlmeier–Degos disease: a narrative review

Sattler

Magro

Shapiro

et al. 2022

Orphanet J Rare Dis

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Introduction Kohlmeier-Degos (K-D) disease is a rare obliterative vasculopathy that can present as a benign cutaneous form or with potentially malignant systemic involvement. The gastrointestinal tract is most frequently involved in systemic disease and mortality is often related to bowel perforations. Herein, we provide information to providers and patients regarding gastrointestinal K-D symptomology, pathology, treatment, and diagnosis, with a focus on the importance of timely diagnostic laparoscopy. We present three new cases of gastrointestinal K-D to highlight varying disease presentations and outcomes. Body Based on reviewed reports, perforation is preceded by at least one gastrointestinal symptom: abdominal pain/cramping, anorexia/weight loss, vomiting, diarrhea, nausea, gastrointestinal bleeding, obstipation, constipation, and abdominal fullness. Perforation most commonly occurs in the small intestine and often results in sepsis and death. Although underutilized, laparoscopy is the most sensitive and specific diagnostic technique, demonstrating serosal porcelain plaques similar to those on the skin and characteristic for K–D. The combination of eculizumab and treprostinil is presently the most effective treatment option for gastrointestinal K–D. The pathology of gastrointestinal K-D is characterized by an obliterative intimal arteriopathy eventuating in occlusive acellular deposits of mucin and collagen along with an extravascular pauci-cellular sclerosing process resembling scleroderma confined to the subserosal fat. C5b-9 and interferon-alpha are both expressed in all caliber of vessels in the affected intestine. While C5b-9 blockade does not prevent the intimal expansion, enhanced type I interferon signaling is likely a key determinant to intimal expansion by, causing an influx of monocytes which transdifferentiate into procollagen-producing myofibroblast-like cells. Conclusion Prompt laparoscopic evaluation is necessary in any K–D patient with an abdominal symptom to facilitate diagnosis and treatment initiation, as well as to hopefully decrease mortality. Those with gastrointestinal K–D should start on eculizumab as soon as possible, as onset of action is immediate.

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Section: Treatmentmentioning

confidence: 99%

Gastrointestinal Kohlmeier–Degos disease: a narrative review

Sattler

Magro

Shapiro

et al. 2022

Orphanet J Rare Dis

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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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Disseminated Whitish Papules and Scars on a Patient With Bowel Perforation

Cited by 2 publications

References 10 publications

Gastrointestinal Kohlmeier–Degos disease: a narrative review

Gastrointestinal Kohlmeier–Degos disease: a narrative review

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

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