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.     

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.     

Expression of differentiation markers in cultured cells from various thyroid diseases

Metastatic Crohn's disease is a rare complication in Crohn's disease and there have been only several cases of metastatic Crohn's disease involving the penis. We report one such case. A 22-year-old male student developed anal pain and alternative constipation and diarrhea in December, 1985, followed by diarrhea and lower abdominal pain in January, 1986. He was diagnosed as having Crohn's disease of ileocolitis type. He was admitted to our hospital in July, 1987 because of exacerbation of Crohn's disease. He had anal tags. Soon after admission, two red swollen lesions with central ulcer and erosions were demonstrated at the eversion of the foreskin adjacent to coronal sulcus. Histology of the lesions revealed granulomas with epithelioid cells and giant cells. The lesion responded to a topical steroid. Eight cases of metastatic Crohn's disease involving the penis are briefly reviewed.     

Anitis, vulvar edema and macrocheilitis disclosing Crohn disease in a child: value of metronidazole

BACKGROUND: We report a case of oro-genital and perianal Crohn's disease which progressed for 4 years with no digestive involvement in a 12-year-old girl. CASE REPORT: At the age of 8 years, a young girl developed recurrent anal fissures. Voluminous vulvar edema developed at 12 years with fissurar macrocheilitis. There were no digestive signs and the diagnosis of Crohn's disease was obtained on the basis of granulomatous epithelioid infiltration of biopsy specimens (Bauhin valve, anus, vulva). Metronidazole given per os at the dose of 25 mg/kg/day for 6 months led to partial significant symptomatic remission. DISCUSSION: Unilateral or bilateral vulvar edema is highly suggestive of Crohn's disease even if the classical digestive inflammatory signs are absent. Demonstration of perianal lesions (erythema, pseudocondylomatous formations) must not mislead the diagnosis (sexual abuse). Simultaneous granulomatous lesions in the genital and labial regions is exceptional in Crohn's disease. Ileocolonoscopy is indicated in such cases and alone can demonstrate latent digestive inflammatory processes. Different agents have been proposed for treatment of local Crohn's disease skin lesions. It is difficult to evaluate their efficacy due to the spontaneous variability of disease expression.     

Esophagogastric fistula: a complication of Crohn's disease--case report and review of the literature

Esophagogastric fistula formation as a complication of esophageal Crohn's has been reported in only one case in the literature. In addition, only eight cases of esophageal fistulae of any type have been reported in the setting of Crohn's disease. Unlike the more often described superficial, aphthous disease of the esophagus, response of fistulae to medical therapy has been disappointing, and recurrence and progression are likely. Surgery remains the primary modality for refractory disease. The roles of salicylates, antibiotics, immunosuppressive agents, sealants, and intralesional steroid injections have not been well defined. We present a case of severe, refractory Crohn's disease with fistula formation between the esophagus and stomach, and concomitant involvement of the oropharynx, duodenum, terminal ileum, and cecum.     

Crohn's disease of the larynx

Extraintestinal involvement of Crohn's disease is becoming more readily identified. Laryngeal involvement by Crohn's disease, however, has been reported in only five cases in the medical literature. We present the sixth case as well as an analysis of the prior reports.     

Gastroduodenal, ileocolic and perianal Crohn's disease

The authors present a case of Crohn's disease that initially showed common localisations, and later a rare gastro-duodenal involvement. When the definitive diagnosis of the latter was reached there were already signs of obstruction at the exit of the stomach. Treatment with Prednisolone I.M. and Omeprazole was not successful, and surgery was necessary as a last recourse to resolve the obstruction. A theoretic revision of the main clinical, diagnostic, and therapeutic aspects of this rare from of involvement of Crohn's disease is also presented.     

Mucosal changes in the large bowel with endometriosis: a possible cause of misdiagnosis of colitis?

Endometriotic deposits are not uncommon in the large bowel of women. Because the symptoms produced by endometriosis may lead to investigation by colorectal endoscopic biopsy, the aims of this study were to assess the range of mucosal abnormalities that may occur and to determine whether this could represent a significant potential diagnostic problem. We found mucosal changes in eight of 10 cases of colorectal endometriosis; however, the abnormalities (ulceration, gland architectural disturbance, crypt abscess formation, increased inflammatory cell presence, and smooth muscle fibers between glands in the mucosa) were focal and directly related to endometrial deposits. In one case an abnormal colonic biopsy specimen from a patient with endometriosis supported the erroneous clinical diagnosis of Crohn's disease. Comparing a group of women with endometriosis to a group with adenomyosis of the uterus showed that although more women with endometriosis have endoscopic large bowel biopsies, there was no significant excess of biopsy specimens showing inflammatory changes. Our conclusion is that the endometriosis of the large bowel can masquerade as inflammatory bowel disease or ischemic changes and the possibility should be borne in mind, particularly in cases with atypical clinical features or very focal histological changes.     

Granulocytic sarcoma of the female genital tract: a clinicopathologic study of 11 cases

Eleven patients, 13 to 76 (mean, 40) years of age, had granulocytic sarcoma of the female genital tract (FGT) (ovary, seven cases; vagina, three cases; cervix, one case). In nine cases, the FGT involvement was the initial clinical presentation of the disease, and in the other two cases, the FGT involvement was discovered during a relapse of acute myeloid leukemia. The tumors ranged from 0.5 to 14 (mean, 7.5) cm in greatest dimension. Two ovarian tumors were bilateral, and three were green. Microscopic examination revealed a predominantly diffuse pattern of growth, but cords and pseudoacinar spaces were also present focally in several cases. Sclerosis was seen in five tumors and was prominent in one. Prominent myeloid differentiation was readily recognizable on routinely stained sections in three cases, whereas the neoplastic cells in the other cases were primitive with only rare eosinophilic myelocytes. All 11 tumors were positive for chloroacetate esterase, nine of nine were strongly and diffusely positive for lysozyme, eight of eight for myeloperoxidase, seven of seven for CD68, and six of six for CD43. Examination of bone marrow or peripheral blood performed after the diagnosis of FGT involvement revealed acute myeloid leukemia in three of five cases. Two of these patients died of disease, 1 and 16 months after the initial diagnosis, and the third, who received chemotherapy, is alive and free of disease 8 months after the initial diagnosis. One of the two patients with negative bone marrow had recurrent granulocytic sarcoma 30 months after diagnosis and died of sepsis 1 month later; no residual disease was noted at autopsy. The other patient is alive and free of disease 18 months after the diagnosis. One of the four remaining patients with primary FGT involvement who did not have a bone marrow biopsy died of leukemia 24 months later; no follow-up information is available for the other three patients. One of the two patients with a prior diagnosis of acute myeloid leukemia was alive with disease 26 months later; follow-up is not available for the second patient. The diagnosis was often difficult in these cases, the most common problem being distinction from malignant lymphoma, but carcinoma, granulosa cell tumor, and, rarely, other tumors were considered. Immunohistochemical and enzyme histochemical staining were useful in establishing the diagnosis, although suspicion of the diagnosis on examination of routinely stained sections was of paramount importance.     

KLT-based quality controlled compression of single-lead ECG

We report a rare case of orbital metastasis from hepatocellular carcinoma and review previously documented cases of this condition. The clinical, histopathological, and immunohistochemical characteristics of hepatocellular carcinoma metastatic to the orbit are described. Results from histopathological examination and histochemical findings of the orbital mass established the diagnosis. A review of 10 cases of metastatic hepatocellular carcinoma to the eye and orbit disclosed painful proptosis as the most common clinical sign of hepatocellular carcinoma metastatic to the orbit. In 5 (56%) of the 9 cases that had orbital metastasis (including the present case), the diagnosis was made after the patient first was examined with symptoms from the orbital mass. Metastatic hepatocellular carcinoma should be considered as a rare cause of painful proptosis. While patients usually are seen with signs and symptoms of widespread metastatic carcinoma, patients with hepatocellular carcinoma with orbital involvement may be first examined by the ophthalmologist because of the clinical manifestations of the disease, proptosis and pain. Other orbital lesions associated with painful proptosis are discussed briefly.     

Intravascular lymphomatosis--an indolent or aggressive entity?

Intravascular lymphomatosis (IVL) is a rare malignancy characterized by neoplastic proliferation of lymphoid cells within the lumens of arteries, small veins and capillaries. We report four patients with IVL and review the recent world literature, relating to incidence, clinical features and possible therapy. In these cases diagnosis was established coincidentally in one patient after prostatectomy. This patient eventually had central nervous system involvement. In two other patients IVL was diagnosed from skin lesions. In the fourth case the diagnosis was established at post-mortem examination, where involvement of most organs was evident but particularly kidneys, myocardium, gastrointestinal tract and lymph nodes. Therapy was given to three patients, but the disease progressed in two and they both died with evidence of central nervous system involvement, while the third patient has had a good partial response to combination chemotherapy but has relapsed within two months of completing chemotherapy. As evident from our patients and the literature review IVL has a variable clinical course and currently, there appears to be no effective therapy for this rare disorder.     

Ileocolic linitis in Crohn's disease

Intestinal cancer is uncommon in Crohn's disease but the risk of developing such a tumor is increased. Linitis plastica of the small bowel or colon is very rare. We report a case of ileocolonic linitis plastica which occurred 21 years after an ileocecal resection for Crohn's disease. Partial small bowel obstruction in relation with stricture of the preanastomotic loop prompted us to suspect disease recurrence. The tumor was not diagnosed either on preoperative work-up, or during surgery but only on the histological examination of the resected specimen. Palliative chemotherapy with 5 FU and folinic acid was performed. The patient was asymptomatic after a 17-month follow-up. This observation focuses on the clinical signs and course of linitis plastica. It also illustrates the difficulty of tumor diagnosis in Crohn's disease. Malignant transformation must be suspected if signs of active disease re-occur after a lengthy quiescent period.     

Cutaneous Langerhans cell histiocytosis of the genitalia in the elderly: a report of three cases

Langerhans cell histiocytosis (LCH) is a disease with a broad spectrum of clinical presentations. All of the variants have in common the proliferation of cells which are morphologically, biochemically, and immunophenotypically indistinguishable from Langerhans cells. A retrospective study of three elderly patients revealed the unique presentation of cutaneous Langerhans cell histiocytosis limited to the genitalia. These cases produced a diagnostic challenge because of their unusual clinical presentation and their morphological similarity to certain other entities, including extramammary Paget's disease and malignant melanoma, which may also show S-100-positive atypical cells. All three cases showed infiltrates of histiocytic-appearing cells with folded nuclei and moderate amounts of cytoplasm which involved the epidermis, dermis, or both. Immunoperoxidase studies using antibody to S-100, CD1a and CD68 in each case showed positive staining.     

Indications for clonidine in child psychiatry

We describe here the clinical and pathologic features associated with a specific translocation, t(2;13), in alveolar rhabdomyosarcoma. Tumor specimens from 14 patients with a t(2;13)-positive alveolar rhabdomyosarcoma were studied for cytogenetic-clinicopathologic correlations. Three patients had occult primary tumors; nine patients had primary tumors of the trunk (mediastinal, pelvic, or rectal). The presence of the t(2;13) was ascertained from examination of tumor involved bone marrow in ten patients who had widespread metastatic disease at the time of diagnosis. Marrow involvement was so extensive in three cases that they were initially diagnosed as acute leukemia. Response to therapy was poor, with only five patients achieving a complete response. Twelve patients have died of their disease at a median survival time of 6 months from diagnosis and one is living with recurrent disease; only one patient survives free of disease.     

Isolated jejunal Crohn's disease in a young adult presenting as fever of unknown origin

A 27-yr-old man was referred for fever, weight loss, fatigue, and occasional mild epimesogastric pain without diarrhea or vomiting. Laboratory tests were suggestive of an active inflammatory disease but serological, bacteriological, viral searches, markers of autoimmunity, and neoplasia were all negative. The following were also negative: ultrasonography; conventional x-rays; CT scans; esophagogastroduodenoscopy, pancolonoscopy with ileoscopy; cytohistology including duodenum and ileocolon. Empiric antibiotic regimens failed to control the temperature. Small bowel enema disclosed multiple proximal jejunal strictures. Jejunoscopy revealed erythema, friability, linear ulcerations, stenosis, and dilation in the proximal jejunum. Multiple directed biopsies showed inflammatory changes devoid of any specific features. The patient received steroid treatment and his temperature normalized. Six months later, he was readmitted on account of intestinal subocclusion that was managed conservatively. A few days later urgent laparotomy was performed with peritoneal lavage, repair of double perforated proximal jejunal ulcers, and stricturoplasty. Surgical jejunal biopsy confirmed the results of enteroscopic biopsies. The patient is presently without fever, in the absence of steroid treatment. There have been no reports of cryptogenic fever due to isolated jejunal Crohn's disease in the recent literature. Our patient's clinical picture resembled disease as seen in older children and adolescents, in whom it is a difficult diagnosis owing to the absence of diarrhea. In adults with Crohn's disease isolated jejunal involvement represents approximately 1% of cases. A thorough small bowel investigation is warranted in young adults with cryptogenic fever and low serum protein levels, even in the absence of major gastrointestinal complaints.     

Segmental tuberculosis of the colon diagnosed by colonoscopy

Segmental tuberculosis of the colon is a rare clinical entity. In the absence of pulmonary or ileocecal involvement, colonic tuberculosis may be difficult to differentiate from neoplasm or Crohn's disease by symptomatic and radiological means. Colonoscopy and biopsy can, however, establish the diagnosis and prevent operative intervention, as indicated in the present report. A patient with a radiologically demonstrated strictured lesion of the sigmoid colon was found at colonoscopy to have several hemorrhagic transverse ulcers ranging in diameter between 1-4.5 cm. Multiple target colonoscopic biopsies, specifically from the ulcer beds, revealed necrotizing granulomas and acid-fast bacilli. Antituberculous chemotherapy produced remarkable symptomatic, radiographic and endoscopic improvements and averted exploratory laparotomy for the establishment of the diagnosis. This case report points out the importance of colonoscopic biopsy as a useful diagnostic modality in this disease.     

Crohn's ileitis in a patient with longstanding HIV infection

The differential diagnosis of diarrhea in patients infected with HIV is broad, and includes a variety of bacterial, viral, and parasitic pathogens, as well as malignancies including lymphoma and Kaposi's sarcoma. Idiopathic non-specific inflammatory bowel disease rarely occurs in association with HIV infection. A recent case report described a patient with longstanding Crohn's disease who experienced remission of his bowel disease upon infection with HIV (6). The authors inferred that the remission was secondary to a depressed CD4 (T helper) lymphocyte count. We report the first case of Crohn's ileitis developing in a patient with established HIV infection, depressed CD4 lymphocyte count, and no prior history of inflammatory bowel disease. This case raises questions about the role of CD4 cells in the pathogenesis of Crohn's disease.     

Pharyngitis: how can so simple a disease be so complex?

Intestinal non-Hodgkin's lymphomas are a rare complication of long-standing Crohn's disease and generally arise in sites of active inflammatory disease. To our knowledge, we report the first case of an unusual association between ileal Crohn's disease and a diffuse large B-cell non-Hodgkin's lymphoma involving an adjacent mesenteric lymph node but not the intestinal tract. A 22-year-old man was seen for intermittent abdominal pain, vomiting, and severe weight loss that were suggestive of intestinal obstruction. A segmental ileocolonic resection was performed. Gross examination revealed a terminal ileal inflammatory stenosis and enlarged mesenteric lymph nodes. Histologically, terminal ileal Crohn's disease was associated with a diffuse large cell lymphoma localized within one mesenteric lymph node without intestinal involvement. Immunophenotyping performed on deparaffinized sections demonstrated the B phenotype of this lymphoma.     

Role of computerized tomography in the diagnosis of bone disease in multiple myeloma

PURPOSE: To assess the role of CT in the diagnosis and management of multiple myeloma (MM) and to investigate if CT findings can influence the clinical approach, prognosis and treatment. STUDY DESIGN AND PATIENTS: We reviewed the findings relative to 273 MM patients submitted to CT June, 1994, to December, 1996. The patients were 143 men and 130 women (mean age: 65 years): 143 were stage I, 38 stage II and 92 stage III according to Durie and Salmon's clinical classification. All patients were submitted to blood tests, spinal radiography and CT, the latter with serial 5-mm scans on several vertebral bodies. The CT unit was a Philips Tomoscan SR 7000. RESULTS: CT showed lysis foci in some vertebral bodies (4 cases) where conventional radiography had shown only aspecific osteopenia. CT also depicted vertebral arch and process involvement in 3 cases with the vertebral pedicle sign. Moreover, CT proved superior to radiography in showing the spread of myelomatous masses into the soft tissues in a case with solitary permeative lesion in the left pubic bone, which facilitated subsequent biopsy. As for extraosseous localizations, CT demonstrated thoracic soft tissue (1 woman) and pelvic (1 man) involvement by myelomatous masses penetrating into surrounding tissues. In our series, only a case of osteosclerotic bone myeloma was observed in the pelvis, associated with lytic abnormalities. DISCUSSION AND CONCLUSIONS: The role of CT in the diagnosis and management of MM has not been assessed, because this technique demonstrates tumor extent more accurately than radiography but CT findings do not seem to improve the clinical approach and therapeutic management of the disease. Nevertheless, we recommend CT for some myelomatous conditions, namely: a) in the patients with focal bone pain but normal skeletal radiographs; b) in the patients with M protein, bone marrow plasmocytosis and back pain, but with an inconclusive MM diagnosis; c) to assess bone spread in the regions which are anatomically complex or difficult to study with radiography and to depict soft tissue involvement; d) for bone biopsy.     

Transformations of ileo-rectal anastomosis into ileo-anal anastomosis in hemorrhagic rectocolitis. Indications and results

In order to precise the indications and results of this procedure, we assessed 11 cases of transformation of ileorectal anastomosis (IRA) to ileal pouch-anal anastomosis (IPAA) in ulcerative colitis (UC). These 5 men and 6 women had undergone IRA at a mean age of 31 years, 33 months after the diagnosis of UC (range 3-120). Four of these IRA, excluded by an ileostomy, had never been in function: the cause was severe persistent proctitis in 2 cases and anastomotic leakage and peritonitis in 2 cases. The other 7 IRA had been in function during a mean period of 25 months (range 6-45) and were reoperated because of anal sepsis (1 case), low rectal stenosis (1 case), disabling proctitis (4 cases) and rectal dysplasia (1 case). No patient had specific pathologic signs of Crohn's disease. The 11 IPAA were complicated by pelvic sepsis in 3 cases; surgical drainage succeeded in 1 case, but the 2 others needed pouch excision and terminal ileostomy. The diagnosis of Crohn's disease was eventually made in these 2 patients. The 9 patients with functioning IPAA, at a mean follow-up of 40 months (range 12-60), had 5.2 stools per 24 h (range 2-12), 5 patients had no nocturnal stooling, and 6 had a perfect continence. One patient had disabling chronic pouchitis. In conclusion, proctectomy with IPAA is always feasible when a previous IRA for UC had failed or offers poor results, but should be rejected in case of anal involvement, as that may suggest Crohn's disease. This procedure is followed by similar functional results than after primary IPAA.