Metastatic Crohn's Disease Research Articles

Pediatric cutaneous Crohn disease: A case series of 89 patients and review.

Cutaneous (or "Metastatic") Crohn disease (CCD) is a rare and underrecognized disease characterized by cutaneous granulomatous inflammation. We describe patient demographics, clinical characteristics, histology, and treatment of 89 pediatric cases of CCD, including 78 previously reported and 11 new cases seen at four academic institutions. We emphasize the efficacy of biologic mono- and dual therapy. PubMed identified cases using keywords including "metastatic Crohn disease" and "cutaneous Crohn disease". Patients were identified by retrospective review of the electronic health record including histopathologic diagnosis consistent with CCD. Chart review collected demographic, clinical, and histologic data. Most pediatric patients with CCD are male 55% (49/89), present with edema (73/89, 82%) and erythema (47/89, 53%) of the genitals (33/49, 67%), and have intestinal Crohn disease (69/89, 78%). Oral corticosteroids (53/75, 71%) and metronidazole (29/75, 39%) are the most frequently prescribed medications. Of the 17 patients treated with tumor necrosis factor (TNF)-blockade, 94% (16/17) had partial or total clearance. Ustekinumab resulted in clearance of cutaneous disease in two patients (2/3, 67%) and partial clearance in one patient (1/3, 33%). Two cases achieved total clearance with the use of dual biologic therapy defined as the use of two biologic therapies with differing mechanisms of action or the use of a biologic therapy and small molecule inhibitor. TNF blockade is an effective treatment for pediatric CCD, and interleukin-12/23 inhibitors may be similarly effective. Consideration of dual biologic therapy may be useful in pediatric patients requiring discordant therapies for their intestinal and cutaneous CD.

Fistulising skin metastases in Crohn’s disease: a case report and review of the literature

BackgroundMetastatic Crohn’s disease is a rare disorder characterized by various granulomatous skin lesions that occur independently of gastrointestinal tract involvement. However, currently there is no standardized care or specific treatment. Therapeutic approaches include immunosuppressive agents, such as corticosteroids, azathioprine, and monoclonal antibodies targeting inflammatory cytokines like tumor necrosis factor (TNF).Case presentationWe present a case of a 29-year-old western European woman with significant blind ending abdominal subcutaneous fistulas and abscesses, who sought evaluation in the dermatology department. Histological examination revealed multiple epithelioid cell granulomas. There was no evidence of infectious or rheumatologic diseases such as sarcoidosis. The tentative diagnosis was metastatic Crohn’s disease, which was not related to an intestinal manifestation of the disease. The patient responded to infliximab but had to discontinue it due to an allergic reaction. Subsequent adalimumab treatment failed to induce clinical remission; thus, therapy was switched to ustekinumab, resulting in a positive response. Written informed consent for publication of their clinical details and clinical images was obtained from the patient.For our study more than 1600 publications were screened for cases of metastatic Crohn’s disease on PubMed database. 59 case reports with 171 patients were included in the analysis and evaluated for localization, diagnostic and therapeutic approaches, and complications and were summarized in this review.ConclusionThe successful ustekinumab treatment of a patient with metastatic Crohn's disease underscores the potential of this minimally investigated therapeutic option, highlighting the need for future treatment guidelines given the increasing prevalence of such cases.

Ustekinumab or vedolizumab for refractory metastatic Crohn's disease?

Metastatic Crohn's disease is a rare skin manifestation of Crohn’s disease. A variety of treatment options are available for this condition. But there is a lack of clarity regarding the treatment of resistant cases. There is ongoing debate as to which agent should be selected (vedolizumab or ustekinumab) in patients that are not responding to anti-tumor necrosis factor agents. This study identifies a unique patient who has developed a disease (metastatic Crohn's disease) under treatment with vedolizumab. Following treatment with ustekinumab, the patient regressed completely. Therefore, we recommend ustekinumab primarily in metastatic Crohn's disease.

Metastatic Crohn disease revealing vascular embolization: Report of two cases.

Metastatic Crohn disease revealing vascular embolization: Report of two cases.

Metastatic Crohn's Disease Improving with Vedolizumab.

Metastatic Crohn disease responding to vedolizumab.

Unilateral vulval edema as the presenting cutaneous manifestation of Crohn's disease.

SynopsisVulval edema is an archetypal manifestation of metastatic Crohn's disease, which may predate clinical presentation of gastrointestinal Crohn's disease.

Metastatic Crohn Disease with Groin Localization in an Adult Patient.

Metastatic Crohn Disease with Groin Localization in an Adult Patient.

Vulvar Intra-lymphatic Histiocytosis: Non-classical manifestation of metastatic Crohn's disease

Background: Metastatic Crohn's (MC) is classified as granulomatous lesions in a site discontiguous from the gastrointestinal tract. This rare entity often precedes gastrointestinal symptoms, posing a diagnostic dilemma. In girls, non-tender erythema and edema of the labial majora is most commonly cited as indicative of vulvar MC and pathological diagnosis confirms. We describe one case of vulvar intra-lymphatic histiocytosis (VIH) as a non-classical manifestation of MC.

P048 Metastatic Crohn's Disease Debuting with Severe Oral Manifestation and Vulvar Involvement - A Diagnostic Challenge.

Background: Metastatic Crohn's disease (MCD) is an unusual cutaneous manifestation in Crohn's disease (CD), and concomitant oral and vulvar involvement is even more unusual. It can debut with cavity lesions oral such as canker sores, ulcers, lip edema, granulomatosis, dry mouth, abscesses in the salivary ducts, erythema, gingivitis, glossitis, among others, however, simultaneous compromise with several oral lesions and so severe with loss of multiple pieces dental is very rare. Patient in the fourth decade of life with a family history of autoimmunity who debuts with severe oral manifestations with a requirement for extraction of 14 teeth, severe gingivitis, smooth tongue and glossitis, aphthous stomatitis, ulcers, lip edema and angular cheilitis, without clear cause, and in management by the oral pathology group. Associated with this, there was vulvo-perineal compromise with ulcerated, inflammatory, erythematous and infiltrated lesions. It was initially suspected of Behçet's disease, HLA B51 was performed, it was negative, also, negative pathergy test, and no other suggestive systemic findings. A vulvar biopsy was performed with marked edema of the dermis, dilated lymphatics with perivascular and interstitial lymphoplasmacytic infiltrate and noncaseating granulomas, negative for microorganisms. At this level, it was compatible with MCD, without presence of gastrointestinal symptoms and calprotectin levels in stool in normal range. High and low endoscopic studies and capsule endoscopy were performed in small intestine, without alterations, it was managed by dermatology with topical steroids and by dentistry with dental implants. It was considered patient with inflammatory bowel disease (IBD) type CD with severe extraintestinal manifestations (EIM), although it did not present compromise intestinal treatment, it was decided to start treatment with anti-TNF initially with adalimumab developing paradoxical psoriasis, later treatment with infliximab, again with presentation of severe paradoxical psoriasis, for which it was suspended. Cyclosporine was also used as an immunomodulator, presenting intolerable tachycardia. 18 months after these symptoms, she presented episcleritis of the left eye and begins with colicky abdominal pain and average diarrheal stools 5-a-day, it was performed high and low endoscopic studies without alterations and new capsule endoscopic of small intestine documenting Crohn's enteritis involving the duodenum, jejunum and ileum, considering a patient with IBD type CD, with EIM with vulvo-perineal compromise, severe oral involvement and episcleritis. Currently is under management with azathioprine and Ustekinumab, with clinical improvement significant. MCD represents a diagnostic challenge, it can debut without gastrointestinal involvement, and its clinical and histopathological findings simulate other entities. A timely diagnosis is required to seek early benefit in the patient.

Dermatologic Manifestations of Systemic Diseases in Childhood

Dermatologic Manifestations of Systemic Diseases in Childhood

S2404 Metastatic Crohn's Disease: An Unusual Dermatologic Manifestation of Crohn's Disease

S2404 Metastatic Crohn's Disease: An Unusual Dermatologic Manifestation of Crohn's Disease

PA15: Footprints in the snow: a rare case of metastatic Crohn disease of the scalp responsive to adalimumab

PA15: Footprints in the snow: a rare case of metastatic Crohn disease of the scalp responsive to adalimumab

34. Prepubertal Vaginal Bleeding: A Rare Presentation of Prepubertal Metastatic Vulvar Crohn's Disease

<h3>Background</h3> Metastatic Crohn's disease (MCD) is a rare manifestation of inflammatory bowel disease. Vulvar lesions are seen in only 2% of female patients with Crohn's disease (CD) and frequently predate gastrointestinal manifestations in the pediatric population. Subsequently, MCD is a particularly challenging diagnosis in pediatric and adolescent patients with long-term comorbidities and overall decreased quality of life (QOL). <h3>Case</h3> An 8-year-old female presented to her pediatrician with prepubertal vaginal bleeding, described as initially light pink spotting with wiping that progressed to episodes of bright red bleeding that worsened with activity. Her only other complaints were constipation and generalized groin discomfort. Prior work up from her pediatrician included labs which were significant for iron deficiency anemia out of proportion to her described blood loss. Imaging had also been obtained including a normal pelvic ultrasound and a CT scan which showed diffuse mesenteric fat stranding, characterized as nonspecific but possibly secondary to underlying inflammatory process, and prominent lymph nodes noted within the colon. On physical exam, she was Tanner stage I with significant bilateral vulvar edema and erythema without obvious lesions. The urethral meatus was normal, but the vaginal introitus was difficult to visualize in the office due to the vulvar edema. She was also noted to be at the first percentile for weight and zero percentile for height. She was taken to the operating room for further evaluation, and underwent exam under anesthesia (EUA) with vaginoscopy and vulvar biopsies. EUA again showed significant bilateral vulvar edema with no visible lesions; vaginoscopy showed diffuse inflammation of the vagina and cervix but otherwise no lesions or other abnormalities. Vulvar biopsies revealed lymphangiectasia circumscriptum, which has been associated with CD, neoplasia, and hidradenitis suppurativa. Completion of work up with pediatric gastroenterology is pending at time of this submission, but we anticipate confirmation of diagnosis of CD in the next couple of weeks. <h3>Comments</h3> Chronic, untreated MCD can lead to significant scarring, disfigurement, increased morbidity, and overall decreased QOL. Unfortunately MCD manifestations do not consistently correspond with gastrointestinal symptoms or flares, and thus require frequent follow-up and surveillance. Given the rarity of the condition, there is limited data regarding vulvar MCD, and first line treatment remains corticosteroids with compression garments for symptomatic control of vulvar edema. A multidisciplinary approach is essential for these patients, and a high degree of suspicion is necessary for accurate diagnosis and treatment.

Pediatric Metastatic Crohn's Disease

AbstractCrohn's disease (CD) is a chronic, relapsing, idiopathic condition, characterized by granulomatous, transmural inflammation of the gastrointestinal tract, which can affect its entire length, from mouth to anus. Metastatic Crohn's disease (MCD) is a rare form of skin involvement and is defined by skin lesions without contiguity with the gastrointestinal tract. A 9-year-old patient presented with gastrointestinal complaints and gross skin lesions in the vulva and perianal region. The diagnosis of Crohn's disease was made when the patient was 11 years old, after being evaluated by the colorectal surgeon. Treatment was started with a “top-down” approach, with a sustained response for four years. Afterwards, there was a relapse of the skin disease in previously normal areas, without overt symptoms. Treatment consisted of steroids and local infiltration of infliximab, without improvement. A year later, there was a rapid progression of the skin lesions, and the drug changed to adalimumab, also without response and worsening of the skin lesions. The patient was admitted to the hospital and intravenous steroids were initiated, along with surgical debridement of the lesions. After some improvement, ustekinumab was initiated with satisfactory response. Pediatric MCD has an important impact on the patient's quality of life, with influences on growth and social development.

Ustekinumab for the treatment of paradoxical skin reactions and cutaneous manifestations of inflammatory bowel diseases.

Ustekinumab (STELARA), a human monoclonal antibody directed against IL-12 and IL-23, is FDA-approved to treat psoriasis, psoriatic arthritis, Crohn's disease, and ulcerative colitis. Increasing recognition of paradoxical skin reactions induced by older biologic therapies used for inflammatory bowel diseases (IBD), such as, adalimumab and infliximab, has led to the investigation of ustekinumab for the treatment of the cutaneous and gastrointestinal manifestations of IBD. In addition, ustekinumab may show efficacy in treating paradoxical cutaneous reactions to tumor necrosis factor-alpha (TNF-α) inhibitors. A search of the Medline/PubMed database, with additional citations obtained from the references section of relevant articles, yielded 22 articles that were included in this review. Ustekinumab is a safe and effective option for treating the cutaneous manifestations of IBD, such as, metastatic Crohn's disease and pyoderma gangrenosum. It is also an effective treatment for TNF-α inhibitor-induced paradoxical skin reactions, such as, psoriasis that do not remit spontaneously or with conventional treatment. Additional studies should focus on the optimal dosing of ustekinumab for dermatologic conditions beyond psoriasis.

Successful treatment of metastatic Crohn disease with dapsone

Successful treatment of metastatic Crohn disease with dapsone

Metastatic Crohn's disease

Metastatic Crohn's disease

Metastatic Crohn Disease: A Clinicohistological Appraisal From a Tertiary Care Center in India.

Granulomatous dermatitis due to noncontiguous involvement of Crohn disease (CD) of the gut has been described as metastatic CD (MCD). MCD is the rarest form of cutaneous manifestations of CD. This study aims to analyze the clinicohistological features of MCD in a tertiary care center of India. A retrospective review of patients diagnosed clinically and histologically with MCD over past 5 years was performed. Data on cutaneous features, histological findings, and response to treatment were collected. Twelve patients (3 men and 9 women) with a mean age of 29 years were identified. All women had vulval involvement in the form of edema (80%), ulceration (60%), and fistula (20%). Among the 3 men, 2 had perineal and scrotal swelling and ulcer, whereas the third patient presented with leg ulcer. Intestinal CD was already diagnosed in 50% patients (5/10) at cutaneous presentation, whereas it was diagnosed subsequently in 30% (3/10) cases. Histological examination revealed nonnecrotizing granulomatous inflammation in the dermis in 11 patients (92%). Additional histological features included eosinophilic infiltrate (58%), panniculitis (33%), and vasculitis (33%). The patients were treated with various combinations of oral prednisolone, metronidazole, minocycline, azathioprine, and subcutaneous adalimumab with partial relief. MCD shows a wide spectrum of clinical presentation, with anogenital involvement being the most common. Histology reveals nonnecrotizing granulomas in the dermis in majority of the cases. The diagnosis is extremely challenging in patients without gastrointestinal involvement at presentation, and thus, a high index of suspicion is imperative.

Metastatic vulvar Crohn’s disease preceding intestinal manifestations: a case report and short review

Crohn's disease (CD) is a multi-systemic chronic granulomatous inflammatory disease with a constellation of extraintestinal manifestations affecting various organs, including the skin. Metastatic CD (MCD), defined as granulomatous lesions of the skin occurring at sites separate from the gastrointestinal (GI) tract in patients affected with CD, is the least common cutaneous manifestation, and it is occasionally the primary manifestation of the disease. It may precede any symptoms from the GI tract by months or even years, and it often remains undiagnosed. We present the case of a 42-year-old woman with a rare cutaneous manifestation of MCD involving the external genitalia. The cutaneous lesions of the mons pubis and vulva preceded GI manifestations of CD by 2 years. The patient was treated with azathioprine, oral steroids, and antibiotics. Reduction of the cutaneous nodules, discharge, and erythema was achieved, accompanied by relief of GI symptoms. Response to treatment was maintained during a follow-up of 6 months. In MCD, involvement of the external genitalia is exceedingly rare, with few documented cases in the literature. The integration of clinical information, microscopic findings, and exclusion of other cutaneous granulomatous processes is necessary in order to accurately diagnose this rare dermatologic entity and provide an opportunity for successful therapeutic intervention.

Cutaneous manifestations of metastatic Crohn's disease.

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.