HistoryThis section has been translated automatically.
DefinitionThis section has been translated automatically.
Idiopathic, discontinuously and segmentally occurring, chronic inflammatory granulomatous intestinal disease of unclear etiology of the entire gastrointestinal tract with preferred localization of the ileum. The disease can be accompanied by a variety of granulomatous, often ulcerated skin symptoms. Complications are stenoses, fistulas, malnutrition (s.a.Eneritis regionalis).
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Occurrence/EpidemiologyThis section has been translated automatically.
Incidence: 6/100.000 inhabitants/year.
EtiopathogenesisThis section has been translated automatically.
Crohn's disease is probably mediated by autoimmunological processes. Besides genetic influences (mutations in chromosome 12 and 16 are discussed), dietary factors, oral anticonceptives, smoking, the intestinal flora and the permeability of the intestinal wall play a role. A disturbed permeability of the mucosa seems to be etiologically significant. Mutations of the so-called NOD-receptors are thought to play a pathogenetic role. First data that the disease could be caused by bacterial antigens are gaining increasing importance. The main suspect is Mycobacterium avium paratuberculosis (MAP).
Further associations are known, for example with the autophagy factor ATG17L1 and the interleukin receptor IL23R.
ManifestationThis section has been translated automatically.
Usually before the age of 30 with emphasis on the 2nd and 3rd decade of life. In 4-20% of the patients the oral mucosa is involved. No sex preference. In the relatively rare metastatic Crohn's disease, women are in the majority (about 75% of the clients). The average age of manifestation is 34.5 years. Children are an extreme exception (20 cases in world literature).
Clinical featuresThis section has been translated automatically.
Abdominal pain with (mostly non-bloody) mucous diarrhoea, meteorism, constipation, weight loss. Abdominal pain also appendicitis-like or colicky in the right lower abdomen with a slight increase in temperature.
Classification of the clinic according to the Montreal classification:
A: age of manifestation: A1<16Lj; A2 17-40Lj; A3>40Lj.
L: Location: L1=ilium; L2=colon; L3=ileocolonic; L4=upper GI tract
B: Biological behaviour: B1= not structuring/penetrating; B2= stricting; B3 internally penetrating; B3p: perianal pentrating
Skin / skin-near mucous membrane symptoms:
- Fistulas (40%), perianal aphthous ulcerations, anorectal abscesses (25%) - Note: often anal fistulas are the first monitoring signs of regional enteritis!
- Acne-like abscesses on buttocks, lower abdomen and thighs.
- Erythema nodosum, erythema exsudativum multiforme, vasculitis leukocytoclastic, palmarerythema, uncharacteristic pustulosis of the skin.
- Persistent genital edema
- Recurrent or permanent, usually deep, extensive and very painful ulcers in the oral mucosa area (4-20% of Crohn's disease cases) frequency more frequently than in habitual aphthae. They are significantly larger and more painful.
- Chronic, inflammatory swelling of the lips (see below Cheilitis granulomatosa or orofacial granulomatosis)
- Coincidence with Pyoderma gangraenosum is described
- Occurrence of acrodermatitis enteropathica possible
- occurrence of arthritis and ankylosing spondylitis (80% HLA-B27 positive)
- Individual coincident cases of Sweet's syndrome have been described.
- Episcleritis, iritis, concomitant
- Arthritis, ankylosing spondylitis, sacroilitis
- fatty liver, primary sclerosing cholangitis (K83.0)
Growth disorders in children
Malabsorption syndrome with weight loss
intestinal stenoses with signs of (sub)ileus, rarely perforations
LaboratoryThis section has been translated automatically.
HistologyThis section has been translated automatically.
DiagnosisThis section has been translated automatically.
- Typical endoscopic findings: Disseminated mucosal lesions with often segmental involvement and often involvement of the small intestine.
- Histology with epitheloid cell granulomas including all wall layers.
- Small intestine contrast examination according to Sellink with the typical findings of a stenosis in the terminal ileum.
- The floridity can be controlled by the inflammation parameters below the laboratory values (BSG, CRP, alpha2-globulin, fibrinogen).
Differential diagnosisThis section has been translated automatically.
The main clinical differential diagnoses of the most common granulomatous and non-granulomatous skin diseases.
|Disease||Clinical/histological markers||Disease||Clinical signs|
|Tuberculosis||Inflammation, cheese necrosis||pyoderma gangraenosum||ulceration with probably undermined purple margins|
|Sarcoidosis||Non-caustic granuloma||Behcet's disease||Multiple ulcerations, arthralgia, pathergy|
|Melkersson-Rosenthal syndrome||Non-caustic granuloma||Vulvovaginitis/vaginal infections (venereal and non-venereal, bacterial/fungal)||ulceration, nodules, swelling, vaginal discharge|
|Idiopathic granulomatous vulvitis||Non-caustic granuloma||Bartholinitis/Bartholin cyst||Painful swelling of the labia majora|
|Foreign body granuloma||Foreign body granuloma||acuminate condyloma||Exophytic painless lesions|
|venereal lymphogranuloma||Small, non-hardened ulcers or poplar, inguinal lymph nodes||suppurative hidradenitis||Nodular abscess, multiple lesions|
|Syphilis||Solitary painless ulcerations||Rosacea||Pustules, erythema|
|CD1a-positive Langerhans cell histiocytosis with eosinophilia||Shortness of breath, joint pain and arthritis, red bump on the skin, hepatomegaly and dry eyes (asymptomatic in several cases)||Vulva edema / lymphedema||medical history post-radiotherapy, neoplastic lymph vessel obstruction|
|Allergic granulomatous reactions||
Itchy, more generalized rash: Patch test
|Traumatic bleeding||Pushing pain, local heaviness|
TherapyThis section has been translated automatically.
- Therapy of skin and mucous membrane changes requires prior treatment of the inflammatory bowel disease (therapy of first choice: 5-aminosalicylic acid preparations + systemic glucocorticoids.
- TNF-alpha blockers(Infliximab and Adalimumab)
- AK against Interleukin 10
- Vedolizumab (Entyvio®) is an enteric-selective integrin antagonist approved since May 2014 for use in patients with active moderate to severe forms of Crohn's disease and ulcerative colitis. Vedolizumab blocks the adhesion molecule alpha4beta7 integrin (see also integrins).
- Surgical therapy for obstruction, perforation, abscess and fistula formation.
Note(s)This section has been translated automatically.
The term "metastatic Crohn's disease" is defined as a sterile, granulomatous, cutaneous lesion without direct connection to the gastrointestinal tract. On the one hand, the term implies a malignant neoplastic process as the underlying disease, on the other hand it is misunderstood that Crohn's disease is a "non-monotopic" granulomatous systemic disease.
Case report(s)This section has been translated automatically.
- Case 1:
- 13-year-old girl with intermittent occurrence of painful, itchy genito-anal swelling and redness since LJ 4. Various unsuccessful pretreatments due to lesions falsely diagnosed as erysipelas or contact allergic eczema. Last 2-4 times/day occurrence of unformed stools with blood deposits.
- Findings: Slightly reduced AZ, slim EZ and depressive mood. Massive, doughy swelling and reddening of the labia majora and partially of the labia minora with coarse infiltration. In the area of the rima ani with preference of the perianal region an erosive, partly coarsely infiltrated, tumorous tumor-like efflorescence with greasy coatings and inflammatory component was observed.
- Laboratory: Accelerated SPA, moderate CRP elevation, leucocytosis at 13.000/µl, Hb 10.8 g/dl, reduced corpuscular volume, markedly decreased ferritin level, ASL titre at 1157 U/ml.
- Colonoscopy: Floride Crohn's colitis, histology of the terminal ileum with distinct lymphofollicular hyperplasia, severe chronic inflammatory signs of epithelioid-cell granulomas in the coecum and rectum and fissure necrosis.
- Histology of the skin: Chronic lymphedema with fibrosclerosis and abundant ectatic lymphatic vessels. In the perivascular area macrophages including some giant histiocytic cells and accumulations of epithelioid-cellular granulomas associated with vessels were found in addition to a loose lympho-plasmacellular infiltration.
- Therapy and course: Initiation of therapy with mesalazine and metronidazole (3 times/day 200 mg p.o.); reduction of stool frequency and healing of genital findings. Continued close outpatient care. Recurrence after 2 years. Combination therapy of corticosteroids (prednisolone 50 mg/day p.o.) and metronidazole (dosage see above). Improvement of findings after 3 weeks; continuation of therapy by the pediatric clinic.
- Case 2:
- 32-year-old female patient with painful mucosal lesions on the palate that have been present for 2 days. The patient's AZ was slightly reduced (fatigue, tiredness, loss of appetite).
- Clinically, an oval, painful ulceration with a diameter of about 0.1 cm was found on the hard palate on the right side. In the area of the soft palate several highly inflammatory "aphthoid" areas about 0.3 cm in diameter. The gingiva, periodontium and the remaining mucosal areas were clinically o.B. A dentogenic cause of the palatal mucosal lesions could be excluded clinically and radiologically.
- Histology of the oral mucosa: Severe acute ulcerative stomatitis with lymphocytic infiltration and few giant cells within the mucosal extravasations; no complete formation of typical epithelial cell granulomas.
- The targeted general examination revealed a previously unknown and also currently clinically (gastro-energeologically) not active Crohn's disease. Immediate systemic therapy with prednisolone for 5 days and local analgesic irrigation with Bepanthen and subcutin solutions alternately led to complete remission within a short time.
LiteratureThis section has been translated automatically.
- Crohn BB, Ginzburg L, Oppenheimer GD (1932) Regional ileitis. A pathologic and clinical entity. J Am Med Ass 99: 1323-1329
- Dalziel TK (1913) Chronic interstitial enteritis. Br Med J 2: 1068-1070
- Djawari DJ (2004) Vulvitis granulomatosa in Crohn's disease. Akta Dermatol 30: 77-80
- Inohara N et al (2003) Nods: Intracellular proteins involved in inflammation and apoptosis. Nat Rev Immunol 3: 371-382
- Kalmar JR (1994) Crohn's disease: orofacial considerations and disease pathogenesis. Periodontol 2000 6: 101-115
- Kugathasan S et al (2003) Dermatologic manifestations of Crohn's disease in children: response to infliximab. J Pediatr Gastroenterol Nutr 37: 150-154
- Lesniowski A (1903) Przyczynek do chirurgii kiszek. Medycyna (Warsaw) 31: 460-464, 483-489, 514-518.
- Mirza MM et al (2003) Genetic evidence for interaction of the 5q31 cytokine locus and the CARD15 gene in Crohn disease. Am J Hum Genet 72: 1018-1022
- Najarian DJ et al (2003) Connections betwen psoriasis and Crohn`s disease. JAAD 48: 805-821
- Saalmann R et al. (2009) Orofacial granulomatosis in childhood - a clinical entity that may indicate Crohn`s disease as well as food allergy. Acta Paediatr 98: 1162-1167
- Tan MH et al (2001) Improvement of Pyoderma gangrenosum and psoriasis associated with Crohn`s disease with anti-tumor necrosis factor alpha monoclonal antibody. Arch Dermatol 137: 930-933
- Hambardzumyan M et al (2016) Granulomatous conditions of the vulva. Gynecologic Dermatology. JP Medical Ltd: 121-128
Incoming links (50)Acrodermatitis enteropathica; Amicrobial intertriginous pustulose; Anal carcinoma; Anal fissure; Antisense molecules; Aphthae habituelle; Aphthae habituelle; Autoimmune diseases; Bile acid loss syndrome; Blue syndrome; ... Show all
Outgoing links (20)Acrodermatitis enteropathica; Adalimumab; Aphthae habituelle; Cheilitis granulomatosa; Cutaneous tuberculosis (overview); Erythema multiforme; Erythema nodosum; Erythema palmare et plantar symptomaticum; Infliximab; Integrins; ... Show all
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