Erythrokeratoderma Variabilis Responding to Low-Dose Isotretinoin

Abstract:  A 2-year-old male child was diagnosed with erythrokeratoderma variabilis, and showed an excellent response to low-dose isotretinoin, with remarkable improvement in all the affected areas within just 2 weeks of treatment.     

IgA Nephropathy-Associated Uveitis: A Case Presentation

ABSTRACT

Purpose: To describe a case of bilateral panuveitis in the setting of IgA nephropathy.

Methods: Retrospective review of clinical records, fundus, and optical coherence tomographic (OCT) images, and fluorescein angiography.

Results: A 36-year-old female presented with IgA nephropathy and contemporaneous ocular manifestations of one-year duration. Clinical exam demonstrated bilateral panuveitis, 3+ anterior chamber (AC) cell in the right eye (OD), and 0.5+ AC cell in the left eye (OS). Funduscopic exam demonstrated diffuse yellow drusenoid deposits bilaterally (OU), accentuated on fundus autofluorescence as focal areas of hyperautofluorescence. Deposits correlated with retinal pigment epithelium hyper-reflectivity on OCT, and choroidal hypo-fluorescence on fluorescein angiography. The patient was managed with oral prednisone.

Conclusion: IgA nephropathy is a systemic autoimmune disease that may be associated with uveitis. Immunosuppression with corticosteroids appears to be an effective therapy.     

SUBACUTE HEPATIC FAILURE-A CLINICAL PROFILE

A combined experience of 37 cases of subacute hepatic failure encountered in five major gastroenterology centres over a period of ten years is discussed. Majority (65%) were males with average age of 38 years. Maximum (54%) were in 5th decade. Jaundice (100%), abdominal distention (38.7%), swelling feet (64%), fever (54%), abdominal pain (54%), exhaustion (78.3%) were the major presenting features. Jaundice and ascites were present in all cases. Pedal oedema (78.3%), hepatomegaly (54%), splenomegaly (32.4%) and encephalopathy (27%) were the other important clinical features. Hypoalbuminemia and prolonged prothrombin time were significant laboratory findings in addition to hyperbilirubinemia and elevated ALT and AST. Hbs Ag was detected in 46%. Major complications encountered were renal failure (48.7%), spontaneous bacterial peritonitis (43.2%), other infections (43.2%), encephalopathy (43.2%) and upper gastrointestinal bleed (22%). 54% died during stay in hospital. To conclude subacute hepatic failure is potentially fatal condition.KEY WORDS: Subacute hepatic failure, HBsAg, Jaundice     

Lymphomatoid granulomatosis: evidence that some cases represent Epstein-Barr virus-associated B-cell lymphoma

Lymphomatoid granulomatosis is currently classified as part of a spectrum of angiocentric immunoproliferative lesions. These were initially thought to be of T-cell phenotype, but recent papers have shown that some cases are B-cell proliferations, sometimes associated with Epstein-Barr virus infection. We reviewed the clinicopathological features of 16 patients with pulmonary lymphomatoid granulomatosis, using immunohistochemistry to assess the phenotype of the infiltrate, the polymerase chain reaction to look for immunoglobulin heavy chain and T-cell receptor gene rearrangements, and in-situ-hybridization to look for Epstein-Barr virus infection. In seven of seven cases the atypical lymphoid population was of B-cell phenotype, with four cases showing evidence of either monoclonality or oligoclonality. All seven cases, including those that lacked unequivocal proof of malignancy, behaved aggressively. Epstein-Barr virus RNA was detected in four cases. We conclude that some cases of lymphomatoid granulomatosis are B-cell lymphomas, sometimes associated with Epstein-Barr virus infection.     

Ultrasonographic Evaluation of Urinary Bladder in Normal,Fern Fed and Enzootic Bovine Haematuria‐affected Cattle

A total of 19 adult hill cattle of both sexes were subjected to trans‐rectal ultrasound scanning of urinary bladder to evaluate bladder wall thickness and the presence of space‐occupying lesions. The animals were divided into four groups. Eight apparently healthy hill cattle maintained under standard ration served as control (group I) and the remaining 11 animals were divided into three groups (II, III and IV). Group II animals (n = 8) were fed with different type of ferns which were further divided into subgroups II‐P, ‐D and ‐B and fed with Polystichum squarrosum (n = 2), Dryopteris juxtaposita (n = 2) and Pteridium aquilinum (n = 4) ferns, respectively. The one animal in group III was a natural case of enzootic bovine haematuria (EBH) and the two animals in group IV were natural cases of microscopic EBH fed with Polystichum squarrosum fern. In group I animals, the average bladder wall thickness was 1.45 mm. The delineation of the bladder wall was uniformly smooth and the echo pattern of the bladder was homogeneously black, which was suggestive of clear urine content. In group II (P, D and B) the average bladder wall thickness of the six animals was 1.87 mm and the sonographic features were within normal limit when compared with controls. In two of the animals of group II‐B, the bladder wall was apparently thick (4.36 mm) and there was no intraluminal mass except at one or two focal elevated points. Animals of groups III and IV showed the average bladder wall thickness of 4.86 mm and were characterized by the presence of irregular sessile masses extending into the bladder lumen. The homogenous anechoic area was reduced centrally due to the presence of a hypoechoic soft tissue mass all around the bladder wall. Post‐sonographic urinalysis, biopsy and necropsy of selected cases further confirmed the sonographic findings.