Community health nursing: can being self-employed work for you in home care?

BACKGROUND: Vulvar involvement in Crohn's disease is uncommon. The elementary lesion is usually an ulceration: 44 cases have been reported in the literature. In 20 p. 100 of the cases, vulvar involvement is the only manifestation of the disease. We report 3 new cases of vulvar lesions in Crohn's disease. CASE REPORTS: The first case had vulvar lesions which complicated ileocolic Crohn's disease, in the other two cases there were no digestive manifestations. One patient developed voluminous edema of the right labium. The diagnosis of Crohn's disease of the vulva was suggested by the characteristic lesions, presence of epithelioid and giant cell granuloma at histology examination of the vulva specimen and finally on rapidly favorable course after treatment with sulfasalazine. The ulcers regressed with medical treatment but remission was temporary with recurrence at treatment withdrawal. DISCUSSION: These cases underline the difficulties encountered in establishing the diagnosis of Crohn's disease in patients who develop vulvar ulcerations alone with no signs of digestive disease and emphasize the difficult problem of long-term control.     

Crohn's disease of the prepuce in a 12-year-old boy: a case report and review of the literature

We report a case of Crohn's disease with involvement of the foreskin in a 12-year-old boy. One year previously, on the basis of clinical features (diarrhea with blood, perianal fissures) and histologic examination, a diagnosis of Crohn's disease was made. Subsequently, he developed phimosis and balanitis and underwent circumcision. Sections submitted from the foreskin revealed noncaseating granulomatous inflammation consistent with Crohn's disease. Crohn's disease with involvement of the genitalia is unusual. Only 26 cases including our case have been reported in the scientific literature. We have analyzed these cases with emphasis on gender, age, clinical features, duration of Crohn's disease, and probable mode of spread to the genitalia. Careful examination of sections from genital lesions, including those submitted from the foreskin, is essential to detect small isolated granulomas that may then lead to the diagnosis of inflammatory bowel disease.     

Infantile pyramidal protrusion as a manifestation of lichen sclerosus et atrophicus

BACKGROUND: A perineal infantile lesion previously described as "skin tag/fold" had recently been named infantile perianal pyramidal protrusion. It appears on the perineal median raphe of girls as a pyramidal soft tissue swelling, covered by smooth, red or rose-colored skin. Its pathogenesis is unknown. As in the case of other perianal lesions, knowledge about it is important, as concern about signs of child abuse grows. OBSERVATIONS: Four girls, 2 of them sisters, with infantile perianal pyramidal protrusion were studied. Three of these girls showed subtle clinical evidence of classic lichen sclerosus et atrophicus on first examination. The other girl developed vulvar lesions of lichen sclerosus et atrophicus months after the diagnosis of infantile perianal pyramidal protrusion. All 4 protrusions disclosed histopathological findings diagnostic of lichen sclerosus et atrophicus. CONCLUSIONS: Infantile perianal pyramidal protrusion is, at least in some patients, a peculiar form of lichen sclerosus et atrophicus that can precede other, more characteristic manifestations. We suggest changing the name to the more precise infantile perineal protrusion. Knowledge of this hitherto unrecognized clinical form of lichen sclerosus et atrophicus can help to explain anogenital symptoms and avoid its misinterpretation as a sign of sexual abuse.     

Adrenal hemorrhage complicating ACTH therapy in Crohn's disease

Huge dose ACTH therapy is used in some severely ill patients with inflammatory bowel disease. We report a teenage girl with Crohn's disease who developed an acute abdomen following ACTH therapy. CT revealed the mass to be a hemorrhagic adrenal gland; the opposite adrenal gland was hypertrophied but without signs of hemorrhage.     

Ultrasonographically-guided fine-needle aspiration cytology in the diagnosis of colonic lesions

BACKGROUND: The use of fine-needle aspiration cytology (FNAC) in the diagnosis of colonic lesions was investigated. METHODS: Some 22 patients (median age 71 years) with a colonic lesion identified on abdominal ultrasonography underwent ultrasonographically-guided FNAC using a 21-G needle. The sample was checked immediately by a cytopathologist for adequacy. RESULTS: Eighteen patients had colonic carcinoma; aspiration cytology detected malignant epithelial cells consistent with colonic carcinoma in 17 patients and severely dysplastic cells in one patient. The sensitivity and specificity of ultrasonographically-guided FNAC in the diagnosis of colonic carcinoma was 94 and 100 per cent respectively. The remaining four patients had a diagnosis of ileocaecal tuberculosis, ileocaecal Crohn's disease, and metastatic adenocarcinoma in the liver with no identifiable primary (two patients). One demonstrated granulomata, grew acid-fast bacilli and the patient was treated for tuberculosis. One had inflammatory cells and the patient was found to have Crohn's disease on histology. The remaining two patients had confirmed metastatic adenocarcinoma in the liver on aspiration cytology but suspected colonic lesions were found to be benign on cytological examination and no primary lesion was subsequently demonstrated. There were no complications of FNAC and patients complained of minimal discomfort. There has been no evidence of tumour recurrence with a median follow-up of 12 (range 1-25) months. CONCLUSION: Ultrasonographically-guided FNAC is a valid method for the diagnosis of colonic tumours.     

Group A beta-hemolytic Streptococcus: an unusual etiology of perianal dermatitis in an adult?

BACKGROUND: Perianal dermatitis usually occurs in children between 1 and 8 years of age. We report a sixth case in an adult. CASE REPORT: A 33 year-old woman complained of perianal itching which quickly extended to the vulva and persisted in spite of anti-hemorrhoid and anti-fungal treatments. After 12 days, she had painful defecation, a well demarcated perianal and vulvar erythema and anal collection. Culture from a perianal swab isolated group A beta-hemolytic Streptococcus (ABHS). After surgical treatment of the collection and a 7 days oral amoxicillin regimen, the patient recovered with no recurrence. DISCUSSION: Symptoms of perianal dermatitis are unspecific, leading to delayed diagnosis and underestimated frequency. Symptoms consist in perianal erythema. Swab culture shows ABHS. The mechanism of colonization of perianal area is not well defined. Although treatment with oral penicillin is usually effective, failures and relapses have also been reported. Complications such as deep infection or abcess formation are infrequent. A bacterial swab should be made in patients with persisting perianal erythma.     

Linkage analysis and identification of deletion in Alagille syndrome gene

PURPOSE: There is concern that patients with presumed ulcerative colitis and significant perianal disease may in fact have Crohn's disease. Moreover, prior perianal disease may be an independent factor for poor outcome of the pelvic pouch. The aim of this study was to evaluate the effect of prior perianal disease on pelvic pouch outcome. METHODS: Between 1982 and 1994, 52 of 753 patients (6.9 percent) who had a pelvic pouch procedure were prospectively identified as having perianal disease. Outcome of the pelvic pouch of these 52 patients (Group I) were compared with the outcome of 701 pelvic pouch patients with no prior perianal disease (Group II). The perianal diseases identified in Group I were fissure-in-ano (17), perianal abscesses (13), fistula-in-ano (7), rectovaginal fistula (3), and significant hemorrhoids/skin tags (25). Eleven patients (21 percent) had more than one type of perianal disease. Twenty-seven patients (52 percent) required a total of 33 perianal operations for the different anal pathologies. RESULTS: Both groups were comparable for the following characteristics: age at time of pelvic pouch procedure, pathology (ulcerative colitis or indeterminate colitis), design of pouch, and type of ileoanal anastomosis (handsewn or stapled). An ileoanal anastomosis leak developed in 21 percent of patients (n = 11) in Group I vs. 11.4 percent (n = 80) in Group II (P < 0.05). Perianal postoperative complications occurred in 11.5 percent of patients (n = 6) in Group I vs. 1.7 percent (n = 12) in Group II (P < 0.05). Total pouch failure rate was not significantly different between the two groups (11.5 vs. 7.6 percent; P > 0.05). Crohn's disease was subsequently diagnosed in 1.9 vs. 2.7 percent (P > 0.05). Subgroup analysis of Group I patients showed no significant difference in outcome according to type of perianal lesion or a history of perianal surgery. CONCLUSION: Prior perianal disease significantly increases the risk of developing an ileoanal anastomotic leak and postoperative perianal complications. However, a pelvic pouch procedure may be an acceptable surgical alternative for selected ulcerative colitis patients with prior perianal disease because the overall pouch failure rate is not significantly increased.     

Incidence of inflammatory bowel diseases in the department of Puy-de-D?me in 1993 and 1994. EPIMICI. Epidémiologie des Maladies Inflammatoires Cryptogenetiques de l'Intestin group

The aim of this prospective epidemiological study was to investigate the incidence of Inflammatory Bowel Disease in the Puy-de-D?me county using the same methodology as EPIMAD's registry. METHODS: From 01/01/93 to 31/12/94, each gastroenterologist (n = 22) collected patients consulting for the first time with clinical symptoms compatible with inflammatory bowel disease. Data were reported on a questionnaire by an interviewer practitioner. The final diagnosis of Crohn's disease and ulcerative colitis was made in a blind manner by two expert gastroenterologists and recorded according to the Calkin's criteria as definite, probable, or possible diagnosis, or unclassifiable chronic colitis or acute colitis. RESULTS: 167 new cases were identified: 112 (67.1%) inflammatory bowel disease for the combined group of definite and probable cases with 79 Crohn's disease (70.5%), 29 ulcerative colitis (25.9%) of which 11 ulcerative proctitis (37.9%), 4 unclassifiable chronic colitis (3.6%) and 55 acute colitis (32.9%). The crude and age-adjusted incidence (per 10(5)/year) was respectively 6.6 and 5.7 for Crohn's disease and 2.4 and 1.9 for ulcerative colitis. The highest age-specific incidence rate for Crohn's disease was between 40-49 years (14.1) and for ulcerative colitis between 80-89 years (6.8). The female/male ratio was 0.8 for Crohn's disease and 1.1 for ulcerative colitis. The median age at the time of diagnosis was 42.6 years for Crohn's disease and 35.3 years for ulcerative colitis. CONCLUSIONS: These preliminary findings revealed a high incidence of Crohn's disease and low of ulcerative colitis in this county. However, these results must be managed carefully because these data were recorded only on two years and the inflammatory bowel disease classified possible and acute colitis require a follow-up.     

Glomerular nephropathy with IgA mesangium deposits and Crohn disease

BACKGROUND: Renal disease is an unfrequent extraintestinal manifestation of chronic inflammatory bowel disease. CASE REPORT: A 12-year-old girl suffered from recurrent abdominal pain, diarrhea and growth impairment due to Crohn's disease of ileocaecal region. After six months of nutritional rehabilitation, an ileo-caecal resection was performed because of intestinal stenosis. The surgical procedure was followed by parietal abcess and cutaneous fistula. One year later, a purulent secretion came out of the fistula associated with fever, hematuria and acute renal failure. Renal biopsy confirmed IgA nephropathy. The course was favorable under parenteral nutrition and after surgical closure of the sigmoido-cutaneous fistula. The microscopic hematuria only persisted but the nephropathy did not relapse even during a further digestive exacerbation. CONCLUSION: IgA nephropathy has been reported in association with chronic inflammatory bowel disease. Its mechanism remains unclear: increased mucosal IgA production in inflammatory bowel, increased serum IgA and/or immune complex deposition in the renal mesangium appear the most relevant hypotheses.     

Role of leukocytes in the pathogenesis of trophic venous disorders]

Although intraoral involvement in Crohn's disease (CD) is observed in only approximately 9% of cases, oral inflammation precedes intestinal symptoms of CD in about 60% of these patients. We describe a 20-year-old male with recurrent, painful, intraoral lesions who presented no other signs of systemic disease apart from severe loss of body weight. From the routinely screened serological parameters only the erythrocyte sedimentation rate and the acute phase reactants were elevated. A biopsy from the vestibular mucosa revealed a dense mononuclear infiltrate and, focally, small noncaseating granulomas suggestive of CD. Gastrointestinal endoscopy was performed showing mucosal involvement reaching from the esophagus to the descending colon. The diagnosis of active CD was confirmed by histopathology of intestinal biopsy specimens. As oral lesions are sometimes treated without a definite diagnosis, we emphasize the need to search for underlying systemic illness in the differential diagnosis of recurrent inflammatory lesions of the oral cavity.     

Colonic involvement in ileal Crohn's disease

OBJECTIVES: To determine the risk and predictive factors for colonic extension in patients with ileal Crohn's disease. METHODS: One hundred and fifty patients with ileal Crohn's disease and no specific colonic lesions on initial colonoscopy were studied retrospectively (median follow-up: 51 months). RESULTS: Twelve patients (8%) developed colonic lesions. Ten-year cumulated risks (95% confidence interval) for colonic extension were 17.2% (range: 5.8-28.6) in the whole group, and 22.4% (range: 8.7-36.1) in the group of 86 patients with repeated colonoscopy. Young age at diagnosis was the only factor predicting colonic extension. Seven patients with colonic extension required immunosuppressive therapy but none underwent surgery. CONCLUSION: Ileal Crohn's disease has a low tendency for colonic extension. Colonic extension has no major prognostic implications.     

Advancement sleeve flaps for treatment of severe perianal Crohn's disease

BACKGROUND: Traditionally proctectomy has been the treatment for severe, complex fistula in ano from Crohn's disease. However, based on the success of rectal advancement flaps in Crohn's disease, circumferential transanal sleeve advancement flaps (TSAFs) were proposed for this subgroup of patients with severe fistula. METHODS: From 1991 to 1995, 13 patients (12 women) with severe perianal Crohn's disease and multiple fistula tracts underwent a TSAF procedure. Data were collected retrospectively using a standard data sheet. RESULTS: There were no postoperative deaths or major morbidity. One year after surgery, the fistula had healed in eight of 13 patients (with three requiring additional surgery before healing). Of patients in whom the procedure failed, three underwent proctectomy for progression of disease and the other two had recurrence of a rectovaginal fistula 6 and 8 months after surgery. Of six variables evaluated (previous procedure, steroid use, steroid dosage, associated Crohn's disease, associated procedures and diverting stoma), only associated procedures were significantly related to a successful outcome (P=0.008). CONCLUSION: Some patients with severe perianal Crohn's fistula and a relatively normal rectum can be offered TSAFs. Even with successful outcome in eight of 13 patients, this may still be a viable option if the only alternative would be total proctocolectomy and a permanent stoma.     

Severe colitis in mucoviscidosis

We report the association of severe indeterminate colitis with cystic fibrosis in a 21 year old woman, with mild pulmonary involvement, and without digestive or pancreatic symptoms or pancreatic enzyme preparation. Ten cases of inflammatory bowel disease associated with a cystic fibrosis have been reported. Most fit with the diagnostic criteria of Crohn's disease. Although this case was compatible with this diagnosis, we have retained the diagnosis of "severe indeterminate colitis" because of the lack of specific histological features of Crohn's disease. The association between inflammatory bowel disease and cystic fibrosis is probably not fortuitous, although the pathophysiological link between the two diseases is unknown.     

Factors influencing outcome and length of stay in a stroke rehabilitation unit. Part 2. Comparison of 318 screened and 248 unscreened patients

Amyloidosis associated with Crohn's disease was found in 7 patients among 85 subjected to intestinal resection for granulomatous enterocolitis. Most of the patients had symptoms of inflammatory bowel disease of relatively short duration before the diagnosis of amyloidosis was made and were without suppurative complications. Systemic involvement was seen in 6 of the patients. One died postoperatively from renal failure, and in 2 other patients kidney transplantation was performed because of deterioration of a pre-existent renal insufficiency. Six patients were alive 6 months to 10 years after amyloidosis was diagnosed. There is great risk of rapid deterioration of kidney function postoperatively in these patients. However, our experience suggests that in some cases the progression of amyloidosis may be delayed or even brought to a halt after surgical treatment of Crohn's disease.     

Direct detection of Mycobacterium tuberculosis complex and M. avium complex in tissue specimens from cattle through identification of specific rRNA sequences

BACKGROUND: In many cases inflammatory bowel disease is accompanied by extraintestinal manifestations. This results in lowering of live quality. The aim of this study was to gather data retrospectively about initial symptoms, extraintestinal manifestations and course of pregnancy in a large unselected population with inflammatory bowel disease in South Germany. PATIENTS AND METHODS: Data from 1975 to 1989 (392 patients) were analyzed and partially compared with data from 1992 to 1995 (211 patients). RESULTS: Patients with Crohn's disease in average have been 25 years old at the time point of initial symptoms, whereas the age of ulcerative colitis patients was 30 years (p < 0.0001). The number of Crohn's disease patients with a long interval between initial symptoms and diagnosis (> 1 year) was significantly decreased in the second population (50% vs 38%; p < 0.05). Dominant initial symptoms in Crohn's disease were indisposition, abdominal pain and nonbloody diarrhea in contrast to ulcerative colitis which manifested mostly with bloody diarrhea. Extraintestinal manifestations occurred in 76% of patients with Crohn's disease and 64.6% with ulcerative colitis. Complications during the course of pregnancy have been detected in 40.5% in Crohn's disease and 60% in ulcerative colitis. CONCLUSION: A better knowledge of initial symptoms and extraintestinal manifestations in inflammatory bowel disease can help to decrease the interval between initial symptoms and the diagnosis. Pregnancy in patients with inflammatory bowel disease needs to be treated with special care.     

Incidence of inflammatory bowel disease in northern France (1988-1990)

There were no data concerning the incidence of inflammatory bowel disease (IBD) in France. The aim of this study was to investigate the incidence of Crohn's disease and ulcerative colitis in northern France. This prospective population based study was realised through the gastroenterologists of the region Nord-Pas de Calais and the Somme Department. Each gastroenterologist referred patients consulting for the first time with clinical symptoms compatible with IBD. Data were collected by an interviewer practitioner present at the gastroenterologist's consulting room. Two independent expert gastroenterologists assessed each case in a blind manner and made a final diagnosis of Crohn's disease, ulcerative colitis, ulcerative proctitis, or unclassifiable chronic colitis. From 1988 to 1990, 1291 cases of IBD were recorded: 674 (52%) Crohn's disease, 466 (36%) ulcerative colitis including 162 proctitis (35%), and 151 (12%) unclassifiable chronic colitis. The mean annual incidence was 4.9 per 100,000 for Crohn's disease and 3.2 for ulcerative colitis. The sex ratio F/M was 1.3 for Crohn's disease and 0.8 for ulcerative colitis. The highest age specific incidence rate for Crohn's disease was between 20 and 29 years: 13.1 for women and 9.8 for men. The highest age specific incidence rate for ulcerative colitis was between 20 and 39 years: 5.5 for women and 6.5 for men. This first French prospective study has shown an incidence rate for Crohn's disease comparable with that seen in north European studies but lower than that seen for ulcerative colitis. These results could be related to the different environmental factors or the genetic background of the population studied, or both.     

MRI appearances in amoebic granulomatous hepatitis: a case report

Amoebiasis is a common cause of liver disease usually presenting as single large or multiple smaller abscesses. Cases with granulomatous hepatitis have rarely been described. We report the case of a 7-year-old girl with amoebic granulomatous hepatitis in which multiple liver abscesses were demonstrated by MRI. A total of 14 abscesses were identified, ranging from 5 mm to 3 cm in diameter. The largest lesions appeared to T2-weighted images as heterogeneous, low-intensity areas surrounded by a double-layered wall, the inner layer of which was hyperintense and the outer layer hypointense. These signs, which have never been described in classic amoebic abscess, represent, we believe, a pattern of hepatic granulomatous amoebiasis lesions. We suggest that MRI should always be performed in cases of amoebic infection.     

Carcinoma arising in anorectal fistulas of Crohn's disease

PURPOSE: Anorectal fistulas are commonly associated with Crohn's disease. Carcinoma arising in an anal fistula in the absence of Crohn's disease occurs rarely. Carcinoma arising in an anorectal fistula of Crohn's disease is likewise rare and is the subject of this article. METHODS: We have seen eight cases in seven patients. Four of these were squamous carcinoma and three were adenocarcinoma. Details of these seven patients are presented. RESULTS: Two deaths in the four patients with squamous carcinoma and one in the two patients with adenocarcinoma with adequate follow-up suggest a poorer prognosis in both types of malignancy than when these lesions occur without Crohn's disease. CONCLUSION: Carcinoma does arise in the midst of the anorectal fistulas and abscesses of Crohn's disease. Carcinoma arising in a Crohn's disease fistula can be very difficult to diagnose. Examination may be limited by pain, stricture, or induration of the perianal and perineal tissues. Examination under anesthesia can also overlook the lesion. Diagnostic examination under anesthesia yields increases with biopsies or curettage of the fistulous tracts.     

Crohn's disease in the elderly

Primary Crohn's disease in the elderly is a not frequent pathology, which however have distinguished from the reheightening or relapse of an inflammatory illness risen up in juvenile age. The Authors compare the personal experience (6 cases on 120 patients operated for Crohn's disease) with the data of the literature. The more frequent localization stays the ileo-colic one, also if in the elderly present better impact the cases of Crohn's colitis (pancolitis or left colitis), with frequent and- perianal implications. The symptomatology is not pathognomonic and above all in the cases of colitis sets problems of differential diagnosis with other pathologies, among which the diverticular disease, that on the other hand, can also coexist with the inflammatory illness. A pharmacological treatment is desiderable, but frequently doesn't result effective. The surgical conservative therapy is not always possible and could be necessary resort to maiming interventions, like massive ileal resections or a total proctocolectomy. From the prognostic point of view, the course appears from the beginning or very favorable (with low index of relapse) or tumultuous (with acute manifestations, which require an emergency surgery, wighted by a more elevated rates of mortality and morbidity.     

Severe chronic iron deficiency in a 17-year-old student

A 17 year old male suffered from iron deficiency of undetermined cause for 2 years. Iron substitution was able to correct it for short periods. With the exception of fatigue and recurring abdominal pain attributed to oral iron therapy no further symptoms were present. The physical status on admission was unremarkable. The laboratory detected intestinal disorders, an anemia of the chronic type without evidence for malignancy or renal failure suggested an inflammatory gastro-intestinal disorder. In spite of a twice negative noninvasive test for gluten-intolerance the clinician favored in his differential diagnosis non tropical sprue over inflammatory bowel disease (IBD, Crohn's disease, Whipple's disease). Histopathology of small bowel specimens did not indicate sprue. An ileo-colonoscopy revealed severe ulcerating ileitis and mild chronic colitis. The histologic specimen revealed a severe ileal inflammation with cosinophilia and the colon specimens epitheloid microgranuloma. These findings are highly compatible with the diagnosis of Crohn's disease. Iron deficiency anemia is common in Crohn's disease. In the current case it is due to disturbed iron uptake. Iron deficiency anemia as sole symptom of Crohn's disease is extremely rare.