Transient bullous dermolysis of the newborn. Case report and review of pathogenesis.

A case of transient bullous dermolysis of the newborn is reported. A healthy Hispanic newborn developed bullae during the first two days of life. From age two to four weeks, the lesions healed with milia formation. There was no residual scarring or hypopigmentation. An induced blister showed dermal-epidermal separation with the PAS-positive basement membrane in the epidermal roof. PAS-positive inclusions were present in the cytoplasm of a few basal cells. Examination by electron microscopy showed degeneration of the collagen and anchoring fibrils. There were numerous stellate inclusions in the endoplasmic reticulum of the cytoplasm of the lesional basal keratinocytes.

Nonamyloidotic fibrillar glomerulopathy and recurrent gestational anasarca.

Congo-red-negative microfibrils have been described in various glomerular diseases, some of which have no known etiology. We report the unusual case of a young woman who, over a period of 17 years, developed recurrent gestational anasarca but was asymptomatic between pregnancies except for proteinuria. Her blood pressure and renal function have remained normal over the years. A renal biopsy done 5 years after her third pregnancy showed diffuse mesangial expansion and irregular thickening of the glomerular basement membrane, both caused by the deposition of nonamyloidotic microfibrils. We discuss the differential diagnosis of this case and review the pertinent literature.

Focal segmental glomerulosclerosis. A common entity in nephrotic black adults.

We studied 100 renal biopsy specimens from adults with the primary nephrotic syndrome in an inner city hospital serving mostly black patients and found that 47 had focal segmental glomerulosclerosis. Most of the men presented in the third decade of life, a peak distribution not seen in women. Half of the patients were hypertensive at presentation. Two thirds of the patients had not used intravenous drugs. The addicts were younger than nonaddicts (mean +/- SD age, 27 +/- 4 years vs 35 +/- 13 years), had greater proteinuria (10 +/- 5 g/d vs 6.3 +/- 5 g/d), and exhibited more glomerulosclerosis and tubulointerstitial fibrosis on biopsy. Of the 18 patients (8 addicts) remaining under our care, 4 addicts and 4 nonaddicts became uremic within 3 years. We conclude that even in the absence of drug addiction, focal segmental glomerulosclerosis is a common cause of primary glomerular disease in black adults, in whom it may represent a nonspecific glomerular reaction to injury. The prognosis in the nonaddict may not be different from that in the addict, but more patients need to be studied.

Gallium 67 scintigraphy in glomerular disease.

To evaluate the diagnostic usefulness of gallium 67 scintigraphy in glomerular disease, 45 patients with various glomerulopathies, excluding lupus nephritis and renal vasculitis, were studied. Persistent renal visualization 48 hours after the gallium injection, a positive scintigram, was graded as + (less than), ++ (equal to), and +++ (greater than) the hepatic uptake. Positive scintigrams were seen in ten of 16 cases of focal segmental glomerulosclerosis, six of 11 cases of proliferative glomerulonephritis, and one case of minimal change, and one of two cases of membranous nephropathy; also in three of six cases of sickle glomerulopathy, two cases of diabetic neuropathy, one of two cases of amyloidosis, and one case of mild chronic allograft rejection. The 25 patients with positive scans were younger than the 20 with negative scans (31 +/- 12 v 42 +/- 17 years; P less than 0.01), and exhibited greater proteinuria (8.19 +/- 7.96 v 2.9 +/- 2.3 S/d; P less than 0.01) and lower serum creatinine values (2 +/- 2 v 4.1 +/- 2.8 mg/dL; P less than 0.01). The amount of proteinuria correlated directly with the intensity grade of the gallium image (P less than 0.02), but there was no correlation between the biopsy diagnosis and the outcome of the gallium scan. It was concluded that gallium scintigraphy is not useful in the differential diagnosis of the glomerular diseases under discussion. Younger patients with good renal function and heavy proteinuria are likely to have a positive renal scintigram regardless of the underlying glomerulopathy.

Pemphigus foliaceus-like, immunologically negative dermatosis in a patient with T cell chronic lymphocytic leukemia.

A 56-year-old black man developed a pemphigus foliaceus-like bullous eruption as the initial presentation of chronic T cell lymphocytic leukemia. Histologic examination disclosed superficial acantholysis consistent with pemphigus foliaceus and an infiltrate of atypical lymphoid cells in the papillary dermis. Repeated direct and indirect immunofluorescence studies yielded negative results. Acantholysis could be reproduced in vitro by incubation of normal human skin with the patient's serum and plasma but not with blister fluid. In the following months, the course of the bullous eruption paralleled that of the leukemia.

Prognosis of the nephrotic syndrome in sickle glomerulopathy. A retrospective study.

Of 240 adults with sickle cell anemia seen over 11 years, 12 had the nephrotic syndrome. In 9 (75%) the glomerular lesion, sickle glomerulopathy, consisted of mesangial expansion and basement membrane duplication. Six patients had type IV renal tubular acidosis. Four of the 9 Patients died within 24 months (17 +/- 5; mean +/- SD), while 5 survived 36 months or longer (80 +/- 49); no significant differences were seen between the former and the latter in age, admission serum creatinine and C3 levels, urinary protein excretion, or the frequency of renal tubular acidosis. Chronic azotemia developed in 3 and acute renal shutdown in another 2. Of 22 patients with sickle glomerulopathy (our 9 added to 13 from the literature) 11 died within 2 years. Ten of these (91%) had developed renal failure, compared to only 5 of the 11 (45%) who survived longer than 2 years (p less than 0.05). The 5-year mortality in the general population of sickle cell anemia is 3.75%, and 75% of patients aged 15 years or older survive 18 years or longer. The nephrotic syndrome, most often caused by sickle glomerulopathy, occurs in 4% of patients with sickle cell anemia, leading to renal failure in two-thirds and death in 2 years in half the patients. The development of chronic azotemia correlates strongly with early mortality. The prognosis is much worse than that in the general population of sickle cell anemia.

Appraisal of lupus nephritis by renal imaging with gallium-67.

To assess the activity of lupus nephritis, 43 patients with systemic lupus erythematosus (SLE) were studied by gallium imaging. Delayed renal visualization 48 hours after the gallium injection, a positive result, was noted in 25 of 48 scans. Active renal disease was defined by the presence of hematuria, pyuria (10 or more red blood cells or white blood cells per high-power field), proteinuria (1 g or more per 24 hours), a rising serum creatinine level, or a recent biopsy specimen showing proliferative and/or necrotizing lesions involving more than 20 percent of glomeruli. Renal disease was active in 18 instances, inactive in 23, and undetermined in seven (a total of 48 scans). Sixteen of the 18 scans (89 percent) in patients with active renal disease showed positive findings, as compared with only four of 23 scans (17 percent) in patients with inactive renal disease (p less than 0.001). Patients with positive scanning results had a higher rate of hypertension (p = 0.02), nephrotic proteinuria (p = 0.01), and progressive renal failure (p = 0.02). Mild mesangial nephritis (World Health Organization classes I and II) was noted only in the patients with negative scanning results (p = 0.02) who, however, showed a higher incidence of severe extrarenal SLE (p = 0.04). It is concluded that gallium imaging is a useful tool in evaluating the activity of lupus nephritis.

Benign cephalic histiocytosis.

A 2-year-old boy was seen because of an extensive asymptomatic papular eruption on the face, neck, and shoulders of 18-months' duration. A skin biopsy specimen revealed cellular infiltrates composed predominantly of histiocytes. Electron microscopy of biopsy material disclosed "comma-shaped" inclusion bodies in the cell cytoplasm, consistent with the findings described in benign cephalic histiocytosis. To the best of our knowledge, this is the first report of this clinical entity in the American literature.

Excision of a large pineal region hemangiopericytoma (angioblastic meningioma, hemangiopericytoma type).

A large hemangiopericytoma was located posterocentrally. The patient presented with headaches and papilledema. Surgical management consisted of total gross excision by a combined right occipital transtentorial and right occipitoparietal transfalcine approach under magnification in three stages over a 6-week period. Removal was aided by dilatation of the right temperooccipital horn ("trapped ventricle") and meningioma-like encapsulation allowed separation of the tumor from the brain and surrounding structures after internal debulking of the tumor. Subsequent peritoneal shunting of the trapped ventricular horn and 5000 rads of radiotherapy were given to the patient. She remains well with an inferior quadrantanopsia on the left side more than 1 year after treatment. Meningiomas of the pineal region, the relationship of meningioma to hemangiopericytoma and malignant mesenchymal tumors (sarcomas) are considered in reference to presumed cells of origin.

Cyclophosphamide therapy and interstitial pulmonary fibrosis.

Intersitial pneumonia and pulmonary fibrosis developed in a 72-year-old man during therapy with cyclophosphamide, vincristine, and prednisone. After extensive investigations, including an open lung biopsy, cyclophosphamide appeared to be the cause of the pulmonary disease. Complete disappearance of tachypnea and the pulmonary infiltrates occurred after the discontinuation of cyclophosphamide and the institution of prednisone therapy. We concluded that the diffuse pulmonary disease in this patient was a result of cyclophosphamide therapy. The clinical and pathologic findings in this case and a review of the literature of cyclophosphamide pulmonary toxicity are reported.