Herpetiform pemphigus with characteristic transmission electron microscopic findings of various-sized ballooning vacuoles in keratinocytes without acantholysis.

We report a unique case of a Japanese woman with herpetiform pemphigus (HP) who had IgG autoantibodies reactive with nondesmosomal sites of keratinocytes and presented characteristic transmission electron microscopic (TEM) findings of various-sized vacuoles in keratinocytes without acantholysis. The patient presented with pruritic annular oedematous erythemas with small blisters lining the margins on the trunk and extremities. Histopathological examinations showed intraepidermal blisters with prominent infiltrations of eosinophils. Direct and indirect immunofluorescence tests revealed the presence of in vivo bound and circulating IgG autoantibodies to the keratinocyte cell surfaces. However, enzyme-linked immunosorbent assays for desmoglein (Dsg) 1, Dsg3 and desmocollins 1-3 showed negative results. Immunoblotting using the full-length human Dsg1 recombinant protein showed a positive band. TEM examination showed various-sized vacuoles squashing the nuclei in many keratinocytes, resulting in rupture of the cells. Immunoelectron microscopic examination revealed IgG deposition over the entire keratinocyte cell surfaces, which spared the desmosomes. IgG antibodies were also present on the inside walls of the vacuoles around the nuclei of keratinocytes and on the cell surfaces of infiltrating eosinophils. This patient also had marked eosinophilia and high levels of thymus and activation-regulated chemokine and interleukin-5 in the serum. These results indicated a novel autoantigen on the nondesmosomal keratinocyte cell surfaces and the pathogenesis of bullous spongiotic change with inflammation in HP.

Anti-desmocollin autoantibodies in nonclassical pemphigus.

BACKGROUND: Despite the established pathogenic role of anti-desmoglein (Dsg) antibodies in classical pemphigus, the significance of autoantibodies to another desmosomal cadherin, desmocollin (Dsc) is at present unknown. No consistent immunoassay for immunoglobulin (Ig) G autoantibodies to Dscs has been developed. OBJECTIVES: The aim of this study was to develop reliable assays to detect anti-Dsc autoantibodies. METHODS: We expressed soluble recombinant proteins (RPs) of human Dsc1-3 in mammalian cells and examined sera of various types of pemphigus, including 79 paraneoplastic pemphigus (PNP) sera, by novel enzyme-linked immunosorbent assays (ELISAs) using the RPs. We also performed ELISAs of Dsc baculoproteins and used the complementary DNA (cDNA) transfection method, and compared the results with those of mammalian ELISAs. RESULTS: Through mammalian ELISAs, IgG autoantibodies to Dsc1, Dsc2 and Dsc3 were detected in 16.5%, 36.7% and 59.5% of PNP sera, respectively, and considerable numbers of pemphigus herpetiformis (PH) and pemphigus vegetans (PVeg) sera reacted strongly with Dsc1 and Dsc3. Mammalian ELISAs were highly specific and more sensitive than baculoprotein ELISAs or the cDNA transfection method. Several Dsc-positive sera, particularly PH sera, showed no reactivity with Dsgs. The reactivity of PNP serum and PVeg serum with Dscs was not abolished by pre-absorption with Dsg RPs. CONCLUSIONS: The results of these novel ELISAs indicated that IgG anti-Dsc autoantibodies were frequently detected and potentially pathogenic in nonclassical pemphigus.

Retrospective study of changes in the sensitivity of the oral mucosa: sagittal split ramus osteotomy (SSRO) versus intraoral vertical ramus osteotomy (IVRO).

We investigated changes in the sensitivity of cutaneous points and the oral mucosa after sagittal split ramus osteotomy (SSRO) and assessed the differences between SSRO and intraoral vertical ramus osteotomy (IVRO). The subjects included in this study were 46 patients with mandibular prognathism who underwent IVRO (88 rami) and 30 patients who underwent SSRO (59 rami). An objective evaluation of the neurosensory status of each patient was completed preoperatively and at 1, 4, 8, 12, and 24 weeks postoperatively. Other variables studied for each patient included sex, age, magnitude of mandibular setback, and amount of blood loss during surgery. We found that a neurosensory recovery occurred earlier in the oral mucosa than at cutaneous points. The number of oral mucosa points showing reduced neurosensory function and neurosensory disturbance after SSRO was significantly higher than after IVRO at 1, 4, and 8 weeks (P<0.05). The nerve recovery observed after SSRO was delayed for a longer period than that noted in our previous study of IVRO. In conclusion, we found changes in sensitivity at cutaneous points and the oral mucosa after SSRO and assessed the differences between SSRO and IVRO.

Determining the location of the internal maxillary artery on ultrasonography and unenhanced magnetic resonance imaging before orthognathic surgery.

The purpose of this study was to examine the efficacy of ultrasonography (US) and unenhanced magnetic resonance imaging (MRI) to determine the location of the internal maxillary artery (IMA) before orthognathic surgery. The study subjects were 19 patients (seven males and twelve females) with mandibular prognathism seen at the authors' institution between March 2012 and April 2013. The distance from the skin to the IMA (S-IMA) and the distance from the mandibular notch to the IMA (MN-IMA) were measured. Using the US and coronal MRI images, S-IMA(cl) and MN-IMA(cl) in the closed position and S-IMA(op) and MN-IMA(op) in the open position were measured at a total of four points in each cross-section. There were significant correlations between the distances measured on coronal MRI and US for all groups (P<0.05). A total of 35 (92%) IMAs were classified as clear and three (8%) as unclear based on the US findings. Regarding the location of the IMA, 37 of the 38 sides studied (97%) were of the lateral type, while only one (3%) was of the medial type. The results of this study indicate that US can be used effectively to determine the location of the IMA.

Changes in the sensitivity of cutaneous points and the oral mucosa after intraoral vertical ramus osteotomy.

In this study we investigated the changes in the sensitivity of cutaneous points and the oral mucosa that occur after intraoral vertical ramus osteotomy (IVRO). Additionally, postoperative changes in the sensitivity and the relationships between neurosensory disturbance and factors associated with IVRO operations were evaluated. An objective evaluation of the neurosensory status of cutaneous points and the oral mucosa of each patient was completed preoperatively and at 1, 2, 4, 8, 12, and 24 weeks postoperatively. The other variables studied for each patient included sex, age, magnitude of mandibular setback, and the amount of haemorrhage that occurred during surgery. In addition, the relationships between neurosensory disturbance and factors connected with IVRO operations were evaluated. We found that at cutaneous points, contributing factors such as sex, age, the magnitude of mandibular setback, and haemorrhage were associated with an increased risk of neurosensory disturbance after IVRO. However, these factors were not associated with that in the oral mucosa. In conclusion, we demonstrated the changes that occur in the sensitivity of cutaneous points and the oral mucosa after IVRO, the postoperative changes in sensitivity, and the relationships between neurosensory disturbance and factors connected with IVRO operations.

Osseous healing after a sagittal splitting ramus osteotomy.

The purpose of this study was to examine osseous healing in the cleavage between the bone fragments after sagittal splitting ramus osteotomy (SSRO) utilizing computed tomography (CT). The subjects were 13 patients with mandibular prognathism who underwent bilateral SSRO. CT was used to study the rami immediately after surgery, then 6 months, 1 year, and 2 years after surgery. Many concave type (the angle between the cleavage >90°) anterior borders developed into the stairway type (the angle between the cleavage <90°) 6-12 months after surgery. Few borders changed to the smooth type. Almost all of the stairway and concave posterior borders changed to the smooth type in the 6-12 months after surgery. Regarding the posterior borders 1 year after surgery, the cleavage of distal and proximal bone fragments demonstrated stable osseous healing (smooth type) in cases where the length and width between the bone fragments were large. In conclusion, the authors successfully demonstrated detailed osseous healing in the cleavage between the bone fragments after SSRO. Remodelling between bone fragments is a major mechanism of osseous healing after this procedure.

Dentocraniofacial morphology of 12 Japanese subjects with unilateral cleft lip and palate with a severe Class III malocclusion: a cephalometric study at the pretreatment stage of surgical orthodontic treatment.

OBJECTIVE: The present study clarifies the dentocraniofacial morphology of patients with cleft lip and palate (CLP) with severe Class III malocclusion prior to surgical orthodontic treatment. METHODS: The sample was 12 Japanese male subjects with repaired complete unilateral CLP (surgical CLP group; 21.2 +/- 1.92 years in mean age). Two sets of patients without CLP Class III malocclusion, consisting of 19 male subjects treated by surgical orthodontic treatment (surgical Class III group; 23.4 +/- 6.35 years in mean age) and 14 male subjects treated by nonsurgical orthodontic treatment (nonsurgical Class III group; 18.7 +/- 3.49 years in mean age) were used as controls. Analyses were performed using lateral and posteroanterior (P-A) cephalograms. RESULTS: (1) The surgical CLP group showed significantly smaller values for overjet, SNA angle, and inclination of the maxillary incisor as compared with those of the surgical and nonsurgical Class III controls. The values of SNB, mandibular effective length, and ramus height in the surgical CLP group were significantly smaller than those of the surgical Class III group but were similar to those of the nonsurgical Class III group. (2) The mandible and the upper and lower dental arches deviated laterally toward the cleft side. The displacement of the mandible was correlated with that of the maxilla. These results show that CLP patients who required surgical orthodontic treatment had a characteristic dentocraniofacial morphology, compared to controls without CLP with Class III malocclusion.