Acquired reactive perforating collagenosis: a clinicopathologic study of 15 cases from Saudi Arabia.

BACKGROUND: Perforating dermatoses include a family of disease characterized by transepidermal elimination of collagen. Despite being described many years ago in association with systemic disease, it is still clinically misdiagnosed. OBJECTIVE: A retrospective clinical and pathologic review that will increase clinical and pathologic diagnostic awareness of this entity and explore associated systemic disease. PATIENTS AND METHODS: In this report, we present from Saudi Arabia for the first time details of a series of patients seen at the King Abdulaziz Medical City, WR, Jeddah, Saudi Arabia, during the period 2002-2009. RESULTS: The study revealed 15 histologically confirmed cases of acquired reactive perforating collagenosis (ARPC). Patients were predominantly adult females with a mean age of 54 years. The lesions were pruritic, predominantly multiple, erythematous excoriated papules and umblicated nodules centrally crusted with an average duration of 6 months, involving the extremities with or without the trunk in 12 patients. Associated systemic disease included diabetes mellitus in 12 patients and chronic renal failure on dialysis in 8 patients. The provisional clinical differential diagnosis included perforating collagenosis in only eight patients. CONCLUSION: ARPC is not uncommon in Saudi Arabia. The noduloulcerative lesions involve predominantly the extremities, and diabetes mellitus is the most common associate. It is hoped that this article will increase awareness for recognition of this entity to start appropriate therapy.

6-thioguanine as a cause of toxic veno-occlusive disease of the liver.

Lesions of hepatic veno-occlusive disease were found in the needle biopsy specimen of one patient suffering from chronic granulocytic leukaemia and in the liver at necropsy of a second patient suffering from acute myeloid leukaemia. The treatment included administration of 6-thioguanine which was the only relevant compound used in the first patient and which was combined with cytosine arabinoside in the second patient.

Appendicular schistosomiasis: a cause of clinical acute appendicitis?

The role of schistosomes in the pathologenesis of acute appendicitis in an endemic area was investigated. Of 1600 appendicectomies received in our laboratory, 26 showed appendiceal schistosomiasis, which prompted what we believe to be the first detailed histopathological evaluation of all appendices with schistosomal infestation, without prior knowledge of the clinical and operative diagnoses. The results suggest that there are two types of schistosomal appendicitis, each with distinct clinicopathological features and different pathogenetic mechanisms. They could be called obstructive and granulomatous schistosomal acute appendicitis, respectively.

Extraspinal meningioma. Case report.

A case of ectopic extraspinal meningioma is reported which presented as a tumor at the back of the neck of a 51-year-old woman. This neoplasm was of considerable size and arose extradurally in the region of the C-2 nerve root, with its main bulk lying almost totally outside the spinal canal in the right suboccipital region. Total resection was accomplished in two stages, with no evidence of recurrence after 1 year. The rarity of such cases prompted this report.

Primary hepatocellular carcinoma in the eastern province of Saudi Arabia: treatment with combination chemotherapy using 5-fluorouracil, Adriamycin and mitomycin-C.

An analysis is done of 40 evaluable patients treated with a combination of 5-Fluorouracil, adriamycin and mitomycin-C for primary hepatocellular carcinoma in Saudi Arabia. In only 5 patients (12.5%) objective partial remission was achieved. The duration of remission for responding patients was: 6, 6, 8, 12 and 20 weeks, respectively. Responders demonstrated a short survival time of 8, 8, 10, 15 and 33 weeks. The median survival time of non-responders was 8 weeks, which was not different from that of responders. An analysis of potential factors that might contribute to our poor results is presented.

Tuberculous peritonitis: the value of laparoscopy.

The laparoscopic and pathological diagnoses of 43 patients who underwent abdominal laparoscopy for various indications are presented. Major indications for the laparoscopy included hepatomegaly in 32 patients, ascites in 28, and pyrexia of unknown origin (PUO) in 18 patients. A combination of two or more of these indications was a more common feature. The most frequently encountered laparoscopic diagnoses were tuberculosis and chronic liver disease (16 patients each), followed by cancer (9 patients). However, on pathological examination of peritoneal or liver biopsy tissue and on follow-up, tuberculosis was confirmed in 12 patients, chronic liver disease in 14 patients and hepatocellular carcinoma in 11 patients. No complications were encountered during the laparoscopy. Our findings indicate that abdominal laparoscopy is a safe, quick and inexpensive diagnostic tool, particularly when appropriate and adequate tissue is taken for pathological examination. In such instances, laparoscopy would save an unnecessary laparotomy, especially where tuberculosis and cancer are considered in the differential diagnosis.

Campylobacter pylori in Saudi patients undergoing upper gastrointestinal endoscopy: prevalence and effect of conventional therapy.

Patients with symptoms of gastritis or peptic ulcer disease were recruited to study the prevalence of Campylobacter pylori. On the basis of the endoscopic diagnosis only, the isolation rates of the organisms in normal, gastritis or gastroduodenitis (GD), and peptic ulcer (PU) disease patients, were not significantly different among the 89 patients evaluated. However, analysis based on histopathological evaluation (in 73 out of these 89 patients) revealed a significant difference (p = 0.00016) in the isolation rate between histologically normal individuals (14%) and those with GD (89%). Also, a significant (p = 0.03) difference was observed in the C. pylori detection rate among patients with GD and those with PU disease (61%). After adjustment for multiple comparisons, only the difference in C. pylori prevalence between normal and GD patients was maintained. After conventional therapy, 23 patients who initially had GD or PU disease were submitted to re-endoscopy. In the latter group, a correlation between presence or absence of organisms and histological healing was noted. The potential pathogenesis of C. pylori in gastritis and peptic ulcer disease, and designs for future trials are discussed.

Non-steroidal anti-inflammatory drug-induced gastropathy: a comparative endoscopic and histopathological evaluation of the effects of tenoxicam and diclofenac.

A 4-week double-blind study compared the potential for 20 mg/day tenoxicam or 100 mg/day diclofenac sodium to induce gastropathy in 36 patients with joint disease and assessed the influence of gastric colonization by Helicobacter pylori. Endoscopic assessment at the end of 4 weeks indicated that the mucosa was normal in 79% of tenoxicam-treated patients and 59% of diclofenac-treated patients. Only 5% of patients in the tenoxicam group developed severe gastroduodenitis (> 11 haemorrhages or erosions) compared with 18% in the diclofenac group. Histological evaluation indicated that 58% and 47%, respectively, of tenoxicam-treated and diclofenac-treated patients retained normal mucosa after treatment. Diclofenac treatment was discontinued in two patients, due to a duodenal ulcer or severe erosive gastritis. Overall, 5/14 patients with moderate to severe colonization with Helicobacter pylori developed severe chronic active gastritis or ulceration, compared with the 1/22 patients in whom colonization was either absent or mild (P = 0.02). Tenoxicam and diclofenac did not show major differences in terms of gastrointestinal safety, although the trends favoured tenoxicam. The presence of severe colonization of the gastric mucosa with Helicobacter pylori appears to be an important factor for development of severe gastritis or ulceration.

Pattern of pleural effusion in Eastern Province of Saudi Arabia: a prospective study.

In order to establish the clinical pattern and aetiology of pleural effusion in adults in the eastern province of Saudi Arabia, all patients aged 18 years and above presenting with clinical and radiological evidence of pleural effusion, between 1st December 1987 and 30th November 1991, at three participating hospitals, were prospectively studied. Of the 201 (145 male, and 56 female) patients recruited, 102 (51%) were Saudis; their mean age was 43.4 +/- 17.8 years. Pleural effusion was more common on the right side (56%) than the left (32%). In rank order, the most common diagnoses were tuberculosis (37%), neoplasm (18%), parapneumonia (14%), and congestive cardiac failure (14%). There were a variety of other causes. The aetiology was indeterminate in only 3 (1.5%) patients. Patients with tuberculosis (75 patients) were relatively young (mean age 33.4 years) and 50 (67%) of them were expatriates, mainly from the Indian subcontinent and Yemen. Of the diagnostic procedures, the most useful were histological examination and culture of pleural biopsy. The contribution of culture and cytology of pleural fluid to diagnosis was rather small. It is concluded that the clinical pattern and aetiology of pleural effusion observed in this study are similar to those of the developing countries, although they seem to have been influenced by the large migrant labour force in the country.

The morphological spectrum of tumors of adipose tissue: A pathological analysis of 685 tumors.

Over a seven-year period, 1093 cases of tumors of soft-tissue origin were recorded by the Pathology Department of King Fahd Hospital of the University, Al-Khobar. Of these, detailed histopathological analysis was performed on 685 tumors of adipose-tissue origin removed from 640 patients. Of the 45 multiple tumors, 31 were angiolipomas and 14 were lipomas. Of the total tumors there were 579 lipomas removed from 565 patients, 889 angiolipomas removed from 58 patients, three spindle-cell lipomas, four pleomorphic lipomas, two myelolipomas, two angiomyolipomas, and six liposarcomas. The majority of adipose-tissue tumors occurred in males, for a ratio of 3:1, and dominantly affected patiens in the age range 16 to 60 years (overall mean age, 37 years). The most common site affected was the trunk. The histological subtypes as correlated to their clinical parameters are presented and discussed.

Pediatric liver disease in the eastern province of Saudi Arabia: A clinicopathological study.

This prospective study analyzes the clinical features and histopathological findings in liver biopsies of pediatric patients presenting to the hospital with liver disease during a 10 year period. Only those patients in whom liver biopsy was performed for a tissue diagnosis were included. Fifty patients were investigated, all below the age of 12 years, of whom 36 were male and 14 female. Thirty-two were of neonatal-infantile group, 11 had a diagnosis of neonatal giant cell he hepatitis of infections origin and an intact biliary tree. Two had septic shock and one had leishmaniasis. The remaining 18 patients of the neonatal-infantile group constituted five case of glycogen storage disease, six of infantile obstructive cholangiopathy (biliary atreasia), four of fatty change and one each of congenital hepatic fibrosis, neuroblastoma and nonspecific reactive hepatitis. The eighteen older children had the following diagnoses: thalassemia in five, sickle cell disease in four, two each of Reye syndrome and hepatoblastoma. The remaining were one each of glycogen storage disease, Rotor syndrome, cirrhosis, fatty change and non-Hodgkin lymphoma (NHL). These findings are presented and discussed.

Lipoid proteinosis: Clinical genetic, and pathological study of a Saudi Arabian family.

We present the morphological features and clinical progression of lipoid proteinosis observed in six patients from a Saudi family. The index case presented with hoarseness and characteristic skin lesions. Some of the patients had photosentivity with milia and hypertrichosis. The diagnosis of lipoid proteinosis was confirmed by histopathology and by the findings of normal blood porphyrin levels. The condition is inherited as an autosomal recessive disorderm and consanguinity was an important factor. The rarity of this condition and the common occurrence of close-relative intermarriages in Saudi Arabia prompted this report, which we believe is the first and largest family with lipoid proteinosis possessing an established Arabian ancestry.

Wilson's disease in Saudi Arabia: Report of a Saudi Arab family.

A Saudi family with Wilson's disease (hepatolenticular degeneration) is described. The index case presented with anicteric hepatitis and hydrops of the gallbladder. Neurological involvement appeared later. The diagnosis of Wilson's disease was based on the presence of Kayser-Fleischer rings, a low serum ceruloplasmin level, and an elevated urinary copper concentration. Histological examination of the liver biopsy specimen revealed active cirrhosis. Acute hepatic failure developed during D-penicillamine therapy. Continuation of the drug at a lower dose, along with other supporitve measures, was successful in reversing this. After three years of therapy, the index patient's neurological signs disappeared, and liver function and gallbladder size and function returned to normal. Family screening revealed that three other siblings have the disease, and all have been treated with D-penicillamine. The parents are related but are asymptomatic. An unusual feature of the index case was the presence of a distended nonfunctioning gallbladder that reverted to normal with decoppering. Although D-penicillamine treatment possibly precipitated the acute hepatic failure, paradoxically it was also successful in treating it.

Chronic idiopathic ulcerative colitis in Saudi Arabia: A clinicopathological study of 76 cases.

In this report we present our experience with 76 cases of chronic idiopathic ulcerative colitis (IUC) out of 1279 consecutive colorectal biopsies (6.0%), seen during an 11-year period (1983-1994), in a tertiary care teaching hospital. During the same period, 12 (0.8%) patients with Crohn's disease were seen. Of the 76 patients with IUC, forty-nine were male and 27 were female, with an age range of six to 88 years, a mean of 38 +/- 16 and a median of 36 years. Forty-nine patients were Saudi Nationals (27M, 22F), 21 were non-Saudi Arabs and six were Asians. In most patients, the onset of IUC was at 20 to 49 years (70%). The disease duratio at diagnosis ranged from one month to five years, with a median of 12 months. The follow-up period ranged form 13 months to 11 years, with a median of three years. Diarrhea, hematochesia and abdominal pain were the dominant symptoms. The disease grade was generally of mild to moderate severity (55 patients, 72%) and was of low stage (distal involvement in 69 patients, 91%). The rarity of skin manifestations and of development of colonic cancer is to be noted in spite of the relatively short period of follow-up. Our findings compare with experience from the region, confirming the mild course of the disease as contrasted to Western experience. These findings will be discussed.