Linear immunoglobulin A (IgA) bullous dermatosis of childhood: identification of the target antigens and study of the cellular sources

Summary A 4-year-old girl developed numerous tense blisters on the body. The blisters healed without scarring. Histopathological and immunofluorescence studies showed findings consistent with linear immunoglobulin A (IgA) bullous dermatosis of childhood, Immunoelectron microscopy revealed deposition of IgA in the lamina lucida of the basement membrane zone of the dermal—epidermal junction. Circulating IgA autoantibody was positive at the titre of 1:128 and recognized the antigens located on epidermal sites of 1 mol/l NaCl-split skin. Immunofluorescence staining of cultured normal human fibroblasts and cultured DJM-1 cells (derived from human squamous cell carcinoma of skin) with the patient's sera demonstrated that both of these cells synthesize the antigens in vitro , although fibroblasts produce the antigens more abundantly. When DJM-1 cells were injected intracutaneously into nude mice, the antigens recognized by the sera were present mainly around the tumour cell islands in a linear pattern, while the dermal-epidermal junction of mouse skin was negative, suggesting that epidermal cells may contribute directly to synthesis and deposition of the antigens at the basement membrane. By immunoprecipitation using cultured normal human fibroblasts, the patient's sera could precipitate at least two specific molecules at 100-kDa and 145-kDa molecular weight.     

"Neuro-Behçet's syndrome"–Case report with pathological findings–

A case of Neuro-Behcet's syndrome was reported clinicopathologically. A man of 37 years old at the time of death, which occurred seven and one-half years after the first appearance of stomatitis and six and one-half years after the first neurological manifestations. The illness began with buccal aphthae, erythema of the lower limbs and arthralgia. Thereafter neurological symptoms such as gait disturbance, numbness of the limbs and urinary disorder recurred. Soon afterwards genital ulcers, diplopia, forced crying and laughing succeeded. Neurologically paresis of oculomotor nerve, bulbar palsy, cerebellar signs, pathologic reflex and superficial and deep sensory disturbance below the level of Th₄ were noted. Ocular signs were not observed. Pathologically 1) Gliosed foci of softening with centrally situated cavities 2) Unsharply defined foci of demyelination 3) Perivascular accumulation of fat granule cells. The above mentioned changes were observed in the subcortical white matter, internal capsule, puta-men, midbrain, pontine basis, medulla oblongata and spinal cord. In the cerebral cortex foci of spongio-necrosis were noted rarely. Demyelination was noted in the optic nerve. In general inflammatory perivascular lymphocytic infiltrations remained mild. There was also cerebellar cortical degeneration. The clinical and the anatomical findings were discussed. Comparison with previously reported chronic cases suggests that this combination of a lowd-grade diffuse encephalitis with severe necrotic focal lesion forms the characteristic pathological pattern in the present case.     

Choroid Plexus Tumors: Experience of 10 Cases with Special References to Adult Cases

Choroid plexus tumors (CPTs) are rare intraventricular neoplasms accounting for about 0.3–0.6% of all intracranial tumors. This retrospective study on CPTs presents clinico-pathological features and management strategies based on a 20-year single-institutional experience. This series included 10 consecutive patients with pathologically proven CPTs; 5 choroid plexus papillomas (CPPs), 3 atypical CPPs (ACPPs), and 2 choroid plexus carcinomas (CPCs). Their clinical, radiological, and histopathological features as well as management including follow-up studies were reviewed. The patients included five males and five females, aging from 0 years to 61 years with median of 28 years. The affected site was lateral ventricle in two adults and fourth ventricle in eight patients; four children and four adults. The most common symptoms were gait disturbance and memory disturbance. All the patients underwent craniotomy with total, subtotal, and partial removals achieved in 50%, 40%, and 10% of the patients, respectively. The occurrence of the high grade subtypes was 50% in both the adult and pediatric groups. The Ki-67/MIB-1 index increased across the three histological subtypes, from CPP to ACPP and then to CPC. Adjuvant therapy was administered in three patients. The two patients (one adult and one child) with CPC died of whole central nervous system dissemination. At a median of 62-month follow-up, the other eight patients were alive, with only one patient having recurrence and reoperation. The results demonstrate that gross total resection is usually curative for CPP and ACPP, and adjuvant chemoradiotherapy would be required for CPC and incompletely resected ACPP.     

Re-anatomization of Anterior Eroded Teeth by Stratification with Direct Composite Resin

Gastroesophageal reflux disease (GERD) is a condition where stomach acids are chronically regurgitated into the esophagus and oral cavity, resulting in the irreversible erosion of tooth structure. The dentist often is the first health care professional to identify the affected dentition. Restorative treatment should be accomplished after management of the systemic condition. Dental treatment improves the patient's oral hygiene, reduces thermal sensitivity, prevents pulpal involvement and further abrasion, and esthetics are improved. This article presents a case report where dental erosion was present because of GERD. Conservative dental treatment of the eroded dentition is described, including diagnosis, treatment planning, bleaching, and restorative reconstruction with direct composite layering techniques without any preparation of the tooth structure. After 1 year of clinical service, the restored teeth present an excellent clinical appearance and require no repair or polishing.

CLINICAL SIGNIFICANCE

The treatment of eroded teeth, caused by gastroesophageal reflux disease (GERD), with direct composite resin appears to be a conservative and esthetic procedure that is well accepted by patients. However, this treatment can be carried out only after complete management of the causes of the GERD.