Mamoru Kasada

Junctional Epidermolysis bullosa with Urethral Stricture

We present a case of junctional epidermolysis bullosa (EB) associated with urethral tract involvement. He was a 27-year-old man who had symblepharon of the right eye, hoarseness and urethral stricture in addition to generalized blister formation. Ultrastructural examination of a biopsy skin specimen demonstrated the characteristic morphology of junctional epidermolysis, a form of EB that has not been previously associated with urethral stricture.

Alteration in the density, morphology, and biological properties of eosinophils produced by bullous pemphigoid blister fluid

SummaryBullous pemphigoid blister fluid (BP-BF) was examined for its effects on the density, morphology, and biological properties of eosinophils. Normodense eosinophils (NEo) were prepared from guinea pig peritoneal exudates by Nycodenz density gradient centrifugation. After culturing with BP-BF, NEo were converted into hypodense eosinophils (HEo) in a time-dependent manner. HEo were morphologically different from NEo in that HEo had spheroidal granules each with a lytic crystalloid core and a significantly increased cell volume. These HEo showed an enhanced antibody-and/or complement-dependent helminthotoxic activity to Schistosoma mansoni larvae, amplified chemiluminescence response to opsonized zymosan, and augmented expression of both FcR+ and CR+. These results suggest that BP-BF contains an activity that may not only induce an eosinophil hypodensity as a consequence of increasing cell volume, but simultaneously enhance an eosinophil cytotoxic potential through augmenting cell-surface receptors and receptor-linked oxidative metabolism. In addition, observed tissue accumulation of this activity suggests that eosinophils may be regulated by their phenotypic change in the skin lesions of bullous pemphigoid and be involved in blister formation.

Sialomucin in Paget cells of extramammary Paget's disease

SummaryThe cytoplasmic sialomucin in Paget cells of extrammary Pagets disease was examined by means of a battery of histochemical techniques. The staining methods used involved an electrolyte-Alcian Blue (pH 5.8), periodic acid-Schiff and azure A at selected pH levels. Methylation, saponification, borohydride reduction, acid hydrolysis, and digestion with diastase, neuraminidase (Vibrio cholerae) or chondroitinase ABC, were also employed. The cytoplasmic mucin was found to exhibit positive reaction for the above staining which were variously altered by the chemical modification procedures and diminished in intensity or abolished by digestion with neuraminidase. These results suggest that the cytoplasmic mucin is sialomucin without side-chain substituent in extramammary Pagets disease located in axillary or genital area, and with a substituent at C7 in the disease located in perianal area.