Metastatic Crohn's disease in childhood

Sabbadini, Chiara; Baratto, Claudia; Schena, Donatella; Peroni, Diego; Girolomoni, Giampiero

doi:10.1111/ddg.12861

Cited by 12 publications

(9 citation statements)

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“…A case review by Sabbadini and colleagues identified the labia as the most common presenting location in girls (65%) and the penis or scrotum in boys (78.6%) . Our review had similar findings, with the labia being the presenting location in 86.7% (26/30) of girls and the penis or scrotum in 66.7% (22/33) of boys.…”

Section: Clinical Presentationsupporting

confidence: 77%

“…Finally, case reports have shown success with tumor necrosis factor (TNF)‐alpha inhibitors. Infliximab is often used in children and initially works well, although disease recurs in 33%‐50% of patients within 5 years . Most cases of adalimumab use in cutaneous CD have been described in adults, but Blasco and colleagues describe a child whose cutaneous lesions improved with adalimumab .…”

Section: Treatmentmentioning

confidence: 99%

“…Infliximab is often used in children and initially works well, although disease recurs in 33%-50% of patients within 5 years. 11,15,27 Most cases of adalimumab use in cutaneous CD have been described in adults, but Blasco and colleagues describe a child whose cutaneous lesions improved with adalimumab. 28 In severe cases, clinicians can consider surgical debridement or hyperbaric oxygen.…”

Section: (Continues)mentioning

confidence: 99%

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Cutaneous manifestations of metastatic Crohn's disease

Schneider

Foster²,

Patel

et al. 2018

Pediatric Dermatology

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Metastatic Crohn's disease is a rare cutaneous complication of primary Crohn's disease. It is a granulomatous inflammatory process, similar to the pathogenic mechanism of Crohn's disease, that occurs in sites discontiguous from the gastrointestinal tract. Metastatic Crohn's disease can precede the development of Crohn's disease by months to years, and children are more likely to present with metastatic Crohn's disease in the absence of gastrointestinal symptoms. Given that approximately 30% of individuals with Crohn's disease present in childhood, early recognition of extraintestinal manifestations of Crohn's disease such as metastatic Crohn's disease can aid in timely diagnosis and management of bowel disease. We present data from two pediatric cases of metastatic Crohn's disease recently seen at our institution in addition to the 61 reported cases of pediatric metastatic Crohn's disease in the literature. This review article will focus on the epidemiology, pathogenesis, clinical features, and histology of and treatment options for pediatric metastatic Crohn's disease.

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Section: Clinical Presentationsupporting

confidence: 77%

Section: Treatmentmentioning

confidence: 99%

Section: (Continues)mentioning

confidence: 99%

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Cutaneous manifestations of metastatic Crohn's disease

Schneider

Foster²,

Patel

et al. 2018

Pediatric Dermatology

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“…The 34 previous reported pediatric MCD cases (20 girls, 14 boys) revealed that labial involvement was most common in 65% of girls, and penile/scrotal involvement was most common in 78.6% of boys. 3 Skin manifestations of MCD are erythema, induration and ulcerations, although lichenoid papules, abscess-like lesions, skin tags or isolated lymphedema can occur. 1 MCD in children presents as polymorphous skin manifestations, and based on clinical and histopathological findings, sarcoidosis, mycobacterial or fungal infections, pyoderma gangrenosum and Behc ßet's disease figure among the differential diagnoses.…”

mentioning

confidence: 99%

Metastatic Crohn's disease in childhood: A case report

Saito

Iwata

Nakajima

et al. 2018

The Journal of Dermatology

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Dear Editor, Metastatic Crohn's disease (MCD) is difficult to diagnose in children because they usually present with only genital lesions and not the usual gastrointestinal symptoms. We describe a 10-year-old girl who was examined 3 months after symptom onset for a small vulvar nodule that had shown enlargement despite topical steroid and antibiotic therapy. Upon initial examination, we observed a 60 mm 9 30 mm red-colored elastic hard mass in the right vulvar region, another redcolored abdominal nodule measuring 20 9 15 mm (Fig. 1a), and a perianal skin tag ( Fig. 1b). A biopsy performed on the vulvar lesion revealed dense lymphocytic and neutrophilic infiltrates in the superficial dermis, and multiple deep dermal epithelial granulomas (Fig. 1c,d). No pathogenic microorganisms were observed using immunostaining with periodic acid-Schiff, Gram and Grocott stains. Differential granulomatous diseases including sarcoidosis were distinguished from various examinations including ophthalmologic findings, chest radiography, computed tomography and blood tests. Based on her clinical presentation and histopathological findings, she was diagnosed with MCD without endoscopically observed gastrointestinal involvement. We initiated the p.o. administration of prednisolone (PSL) (20 mg/day), and 5 months later she showed gradual resolution of her skin lesions (Fig. 1e). (a) (b) (c) (d) (e) Figure 1. (a) A red-colored elastic hard tumor measuring 60 mm 9 30 mm is observed in the right vulvar region. (b) A perianal skin tag is observed. (c,d) Histopathological examination of the vulva showing dense dermal lymphocytic and neutrophilic infiltrates in the superficial dermis and multiple deep dermal epithelial granulomas (hematoxylin-eosin, original magnifications: [c] 940; [d] 9100).(e) Skin lesions are observed to have gradually reduced in size and changed to a soft swelling 5 months after administration of oral prednisolone.

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“…Darüber hinaus können reaktive Dermatosen wie Pyoderma gangraenosum und Erythema nodosum sowie Hauterscheinungen durch Mangelzustände auftreten . Kolleginnen und Kollegen aus Verona präsentieren in diesem Heft den typischen Fall eines 10‐jährigen Jungen mit Morbus Crohn, der sich nach fünfjährigem ausschließlichem Darmbefall auf die Schamregion ausdehnte . Im Gegensatz zum Erwachsenenalter manifestiert sich die Erkrankung in der Mehrzahl der kindlichen Fälle sogar primär extraintestinal .…”

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Kinderdermatologie: ein Kleinod unseres Fachgebiets

Hamm

2016

J Deutsche Derma Gesell

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Liebe Kolleginnen und Kollegen, in dieser Ausgabe des Journals der Deutschen Dermatologischen Gesellschaft finden sich gleich drei Fallberichte zu sehr unterschiedlichen Dermatosen des Säuglings-und Kindesalters. Eine Erythrodermie beim Neugeborenen stellt sowohl in diagnostischer als auch in therapeutischer Hinsicht immer eine besondere Herausforderung dar. Die Neonatologen müssen in dieser kritischen Situation, die immer einer intensivmedizinischen Betreuung bedarf, bemüht sein, drohenden Komplikationen wie hohem transepidermalen Wasserverlust mit der Gefahr einer hypernatriämischen Dehydratation, Temperaturinstabilität und Infektionsrisiko adäquat zu begegnen. Dem hinzugerufenen Kinderdermatologen fällt hingegen vor allem die Aufgabe zu, an der Klärung der zugrundeliegenden Ursache mitzuwirken. Ebenso wird er darauf achten müssen, einen möglichen Schaden des Kindes durch differente Externa abzuwenden, die in einem solchen Fall massiv resorbiert werden und einen unkalkulierbaren systemtherapeutischen Effekt haben können. Die Liste der Differenzialdiagnosen kongenitaler und frühmanifester Erythrodermien ist lang und reicht von Infektionen und Infestationen (neonatale Candidose, Skabies) über entzündliche Dermatosen (atopisches Ekzem, seborrhoische Säuglingsdermatitis, Psoriasis), Immundefekte (Netherton-Syndrom, Omenn-Syndrom, Graft-versus-Host-Krankheit), diffuse kutane Mastozytose, Dermatitis exfoliativa und toxische epidermale Nekrolyse bis hin zu verschiedenen Ichthyosen. Letztere manifestieren sich beim Neugeborenen insbesondere unter den Phänotypen des Kollodiumbabys und der kongenitalen Erythrodermie. Eine erst 2004 als Entität beschriebene Ichthyose wird anhand eines eindrucksvollen Falles von Kolleginnen und Kollegen des Leipziger Universitätsklinikums vorgestellt: das Ichthyosis-Frühgeburt-Syndrom (ichthyosis prematurity syndrome) [1]. Typisch für dieses seltene, autosomal-rezessiv vererbte Krankheitsbild ist die Trias aus neonataler Asphyxie, Polyhydramnion-bedingter Frühgeburtlichkeit und Erythrodermie mit verdickter, käsig-desquamativer Haut [2]. Immer findet sich eine periphere Eosinophilie, häufig ist auch das Gesamt-IgE im Serum erhöht. Der molekulargenetische Beweis der klinisch gestellten Diagnose mit Nachweis von Mutationen im Fettsäure-Transport-Protein-4-Gen [3] konnte im vorliegenden Fall aufgrund des fehlenden Einverständnisses der Eltern leider nicht geführt werden. Kollegen aus Klagenfurt erinnern mit ihrem Fallbericht daran, dass ein Herpes zoster gelegentlich auch schon im Säuglingsalter vorkommen kann [4]. In den meisten derartigen

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Metastatic Crohn's disease in childhood

Cited by 12 publications

References 0 publications

Cutaneous manifestations of metastatic Crohn's disease

Cutaneous manifestations of metastatic Crohn's disease

Metastatic Crohn's disease in childhood: A case report

Kinderdermatologie: ein Kleinod unseres Fachgebiets

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