Neonatal pemphigus vulgaris

A male newborn with skin erosions was born to a 32-year-old woman who was under treatment for pemphigus vulgaris that had been diagnosed 16 months earlier. Antibodies to desmoglein (Dsg)1 and Dsg3 were analyzed by enzyme-linked immunosorbent assay. Index values of antibodies to Dsg1 and Dsg3 were 49 (normal index values, <14) and 121 (normal index values, <7), respectively. Those findings concluded a diagnosis of neonatal pemphigus vulgaris. No new vesicles or bullae appeared in the newborn after the birth. Non-corticosteroid ointments produced prompt epithelialization on the erosive lesions. All the eruptions disappeared in 3 weeks. The level of serum anti-Dsg3 autoantibodies when measured at the 76th day was negative (<5).     

Endemic pemphigus vulgaris

BACKGROUND: Investigators from Brasilia, Brazil, observed several patients with a mucocutaneous disease that resembles pemphigus vulgaris clinically and histologically but with epidemiological features of fogo selvagem. Our objective was to characterize antidesmoglein 3 and antidesmoglein 1 autoantibody profiles in these unique patients who reside in Goiania and Brasilia, Brazil, known endemic regions of fogo selvagem. OBSERVATIONS: We performed serological evaluation of 8 patients with a mucocutaneous disease clinically and histologically consistent with pemphigus vulgaris, as well as 27 healthy relatives of patients with fogo selvagem who reside in these endemic areas. Serum samples from all 8 patients bound desmoglein 3 by cold immunoprecipitation and from 6 patients by enzyme-linked immunosorbent assay, while serum samples from 4 patients bound desmoglein 1 by cold immunoprecipitation and by enzyme-linked immunosorbent assay. Antidesmoglein 3 autoantibodies were detected in 4 of 27 healthy donors by cold immunoprecipitation and by enzyme-linked immunosorbent assay, whereas antidesmoglein 1 autoantibodies were detected in 6 individuals by cold immunoprecipitation and in 3 individuals by enzyme-linked immunosorbent assay. CONCLUSION: These findings provide serological evidence of a new endemic variant of pemphigus vulgaris.     

Localized pemphigus vulgaris

A 32-year-old Japanese man was seen because of a crusted and oozing plaque on his scalp of 3 years' duration. Histologically, suprabasal clefts and bullae containing acantholytic cells as well as intercellular IgG and C3 deposits were found by the direct immunofluorescent technique. There were no circulating pemphigus antibodies. The lesion was controlled by the topical application of 0.05% clobetasol propionate ointment and remained localized for the following 2 years.     

Familial pemphigus vulgaris

A case of familial pemphigus vulgaris is described in an uncle and his niece who developed the disease 18 years apart. The man died from pemphigus in 1964. The diagnosis was confirmed histopathologically in both cases, but immunofluorescent microscopic studies were performed only in the woman because the technique was not available in 1964. HLA typing in the woman and her daughter and sister showed A 26, BW 38 and DRW 4 in all of them. Clinical disease did not develop in the other family members.     

Oesophageal pemphigus vulgaris

Symptomatic involvement of the oesophageal mucosa by pemphigus vulgaris is rare. We describe 1 patient who was treated with oral steroids during a blistering phase, when epigastric pain developed. Endoscopy revealed multiple ulcerations all over the oesophagus, but gastroduodenal mucosa was normal. The symptoms disappeared following cimetidine for gastro-oesophageal reflux and increase of steroid dosage. When painful symptoms appear from the upper digestive tract during corticosteroid treatment of pemphigus, the possibility of acantholytic involvement of oesophageal mucosa must be kept in mind. Its implication for the dosage of steroids is opposite that in steroid-induced peptic ulcers. Carefully performed upper gastrointestinal tract endoscopy is helpful in these patients.     

寻常型天疱疮发病机制的进展

寻常型天疱疮是一累及皮肤和黏膜的自身免疫性大疱性皮肤病,在各型天疱疮中最常见.国内外一直在进行其发病机制的研究,目前尚不完全清楚,其中.最有影响的是桥粒芯糖蛋白的补偿学说.随着免疫学、分子生物学的不断发展,提出更多的发病机制,包括抗体介导的细胞凋亡学说、基底细胞塌陷皱缩假说等.这将有利于进一步发现治疗寻常型天疱疮乃至其他自身免疫病的新靶点,指导临床治疗.     

Outcome of pemphigus vulgaris

Background Pemphigus vulgaris can be divided into mucosal, mucocutaneous and cutaneous subtypes. A higher mortality rate has been shown with mucocutaneous involvement. Objectives The aim of this retrospective study was to analyse the association of clinical subtypes of pemphigus vulgaris with remission rates. Patients/methods One hundred twenty‐eight patients with pemphigus vulgaris, treated with prednisolone 2 mg/kg/day plus azathioprine 2 to 2.5 mg/kg/day, were enrolled. The partial and complete remission rates, at the end of the first and second years of treatment, and the number of relapses were compared in the three groups. Results After disease establishment, 71.1% had mucocutaneous, 18.8% had mucosal, and 10.2% had only cutaneous involvement. The mean duration of follow‐up was 53.5 ± 39.6 months. The mean duration it took the mucocutaneous group to reach a prednisolone dosage of 30 mg/day was significantly longer (P = 0.050). Mucocutaneous patients had a significantly lower rate of remission (31.9%) compared with those with only mucosal or cutaneous involvement (48.6%) at the end of the first year of the treatment (P = 0.029). After 2 years, mucocutaneous patients again had a lower remission rate (32.9% vs. 44.5%). Relapses were also more frequent in this subtype. Those presenting with mucosal or mucocutaneous erosions had a higher rate of active disease after receiving treatment for a year compared with those with only cutaneous presentation (66.7% vs. 45%; P = 0.057). Conclusions In mucocutaneous subtype, clinical control was achieved later, and they had a lower rate of remission at the end of the first and second years of treatment. They were also prone to relapses.     

Roentgen ray-induced pemphigus vulgaris

The pemphigus vulgaris lesions first developed in a 70-year-old man as a reaction to superficial X-ray treatment of solar keratoses on the forehead.