Congenital self-healing histiocytosis. Report of two cases with histochemical and ultrastructural studies.

Congenital self-healing histiocytosis (CSHH) is a rare primary skin disorder. Of the two cases in newborn infants reported here, one had numerous widespread lesions while the other had a solitary ulcerating scalp nodule. Both neonates were otherwise healthy; neither exhibited either systemic involvement or involvement of mucous membranes. The findings drawn from the skin biopsies, including histology, S-100 positivity in the majority of the cells, and the presence of Birbeck granules, were indistinguishable from those described in infantile Letterer-Siwe disease (histiocytosis X). However, the benign clinical course, with rapid regression of the nodules in both cases, was diagnostic of CSHH.

The microvascular anatomy of the canine stomach. A comparison between the body and the antrum.

To investigate whether there is a difference in the microvascular architecture between the body and the antrum of the canine stomach, these two locations were compared with respect to microsphere entrapment and the microvascular architecture and diameter in relation to histology by corrosion casting and by intraarterial injection of india ink. There was 63% shunting of 9-micron microspheres in the antrum, but none in the body. Corrosion cast and Indian ink studies showed that in the body there was a single microvascular network of capillaries that appeared to originate from the arterioles in the submucosa and were in close apposition to the epithelial cells of the gastric glands. The diameter of these capillaries was 8.6 +/- 0.2 microns. In contrast, there were two distinct capillary networks in the antrum: a basal and a superficial. The capillaries of the basal network of the antrum originated from the arterioles at the level of the muscularis mucosa and drained into the capillaries of the superficial mucosa. The capillaries of the superficial network had a significantly larger diameter (10.8 +/- 0.4 microns) than those of the basal network (7.3 +/- 0.2 microns). In many instances the capillaries of the superficial network originated directly from the ascending arterioles passing through the basal network. These direct arteriocapillary connections may have permitted the shunting of 9-microns spheres in the antrum.

A randomized prospective trial comparing oral sodium phosphate with standard polyethylene glycol-based lavage solution (Golytely) in the preparation of patients for colonoscopy.

One hundred and two patients were randomized to receive either oral sodium phosphate or standard polyethylene glycol-based lavage solution (Golytely) prior to colonoscopy in order to establish whether the much smaller volume of the former agent enhanced patient tolerance while maintaining or improving effectiveness and safety. Overall, patients found sodium phosphate preparation much easier to complete. In 37 patients who had had a previous colonoscopy prepared with Golytely, 100% of those now receiving sodium phosphate found it easier to complete and over 90% felt it caused less discomfort, compared with their previous experience with Golytely. Colonoscopists, unaware of the type of lavage solution used, scored the degree of colonic cleansing significantly higher for sodium phosphate-prepared colons compared with colons prepared with Golytely. Serial measurements of blood tests and postural pulse and blood pressure changes did not reveal any clinically significant changes in intravascular volume. Hyperphosphatemia was noted with sodium phosphate, but was transient, and no concomitant decrease in calcium was seen. Histological assessment for possible preparation-induced changes revealed no difference between the two agents. Sodium phosphate is significantly less expensive than Golytely. We conclude that, in the group of patients studied, sodium phosphate is a safe colonic cleansing agent that is better tolerated and more effective than Golytely.

X-linked cutaneous amyloidosis: further clinical and pathological observations.

A 10-year follow-up of a family with X-linked cutaneous amyloidosis confirmed no more than streaks or spots of brown pigmentation of the skin in females but much more varied and severe manifestations in males. These included neonatal colitis, infantile diarrhea, recurrent respiratory infections, corneal dystrophy, photophobia, unruly hair with a frontal upsweep, dry skin, and mottled, muddy-brown pigmentation seen first on the inner thighs and spreading diffusely to the buttocks, trunk, and arms. Amyloid was found in the pigmented skin of adults of both sexes but not in children. An autopsy of a 50-year-old man, subject to recurrent pneumonia, confirmed the presence of amyloid in the skin, but it was not found in other organs. Changes in the lungs were those of late-stage diffuse pulmonary fibrosis. The pattern of inheritance is X-linked, but the pathogenesis remains obscure.

Penicillamine-induced elastosis perforans serpiginosa. Tip of the iceberg?

Elastosis perforans serpiginosa (EPS) is now a well-recognized potential complication of long-term penicillamine therapy. By itself, EPS appears to be a relatively innocuous cutaneous side effect of penicillamine. However, suspicion has been raised in recent literature that EPS may represent only a superficial manifestation of more serious penicillamine-induced systemic elastic tissue damage, particularly involving blood vessels. This is a report of a patient with Wilson's disease who was treated with penicillamine for 14 years. She developed EPS, and histologic examination of the skin revealed the characteristic penicillamine-induced "lumpy-bumpy" elastic fibers in the dermis. More important, nonlesional skin showed the same elastic fiber changes. Of greatest significance was the finding of identical elastic fiber alterations in an artery.

Pneumatosis cystoides intestinalis simulating malignant colonic obstruction.

Pneumatosis cystoides intestinalis is an uncommon condition in which pockets of gas occur intramurally in the gastrointestinal tract. The authors describe the case of a 76-year-old man in whom this condition caused low colonic obstruction, simulating a rectal carcinoma. The patient was successfully treated with high-flow oxygen therapy. This form of therapy has not previously been reported in obstructing lesions, but in this case it successfully relieved the obstruction and thus avoided a colostomy that might have been necessary.

Familial cutaneous amyloidosis with systemic manifestations in males.

We describe a family in which two males and seven females have brown pigmentation of the skin. In the females, the type and distribution of the pigmentation mimicked incontinentia pigmenti; in the males, the pattern was reticulate. The histological appearance was the same in both sexes with amyloid deposits in the papillary dermis, melanin in the basal layer, and slight hyperkeratosis. The females were otherwise normal. Both males had thrived poorly as infants but had survived. One had severe gastroenteritis with blood in the stools starting at the age of three weeks followed by seizures, hemiplegia, and developmental delay; the other had recurrent pneumonia throughout life, a urethral stricture, inguinal herniae, and near-blindness from amyloid deposition in the cornea. Five other males in the family had had severe illnesses. Two died of pneumonia by three months. One died at three months from colitis. Both remaining boys had colitis as infants, failed to thrive, and developed recurrent pneumonia from which one died at three years. We think all of these relatives had the same disease carried by a single gene with pleiotropic effects. The most likely form of inheritance is X-linked.

Burkitt's lymphoma of the appendix: report of two cases.

Two cases of primary Burkitt's lymphoma of the appendix are reported. Both children presented with abdominal pain, and the tumors were discovered during routine appendectomy for suspected "appendicitis." The rarity of the lesion and the prognosis are briefly discussed.

Herpes simplex ulcerative esophagitis in a healthy subject.

A 20-year old white male developed a self-limited ulcerative esophagitis followed by a transient vesicular skin eruption due to infection by herpes simplex virus Type I (HSV-I). While herpetic esophagitis has been described previously, this case appears to be unique in that there was no concomitant serious delibitating illness or pharmacological immunosuppression. Herpes simplex Type I should be included in the differential diagnosis of unusual esophageal ulceration in otherwise healthy patients.

Atheromatous embolization to the stomach: an unusual cause of gastrointestinal bleeding.

This is a case report of an 80-year-old female with persistent blood loss from what was initially considered to be "hemorrhagic gastritis." Distal gastrectomy showed extensive atheromatous embolism and resultant ischemic lesions of the stomach--the apparent cause of the chronic bleeding. The patient has remained well without further blood loss during a 15-month follow-up period. While a small number of cases of gastric hemorrhage associated with atheromatous embolism have been reported, they all have been diagnosed at autopsy. This case is unusual in that the lesion was successfully delt with by surgical resection of the involved area.

Duodenoscopy in the diagnosis of upper gastrointestinal disease.

Duodenoscopy was undertaken in 143 patients with upper gastrointestinal tract symptoms. There were no complications of the procedure. The duodenum was successfully entered in 98% of attempts, and in 90% of patients the examination was judged to be technically successful. When compared with radiological assessment, the findings at duodenoscopy significantly altered diagnosis and management in 21% of patients. The endoscopic appearance of the non-ulcerated duodenal mucosa could not be consistently correlated with changes found in biopsy tissue obtained under direct vision. Duodenoscopy is a valuable adjunct in the clinical assessment of upper gastrointestinal tract symptoms suspected to be due to pathological changes in the duodenum.

Possible pathogenesis of motility changes in diffuse esophageal spasm associated with gastric carcinoma.

A theory is proposed according to which diffuse esophageal spasm associated with carcinoma may be secondary to neural damage due to invasion by carcinoma without obstruction of the esophageal lumen. A case history is presented to support this theory.