Park Sang Gun, Chang Jae Yong, Cho Young-Hun, Kim Soo-Chan, Lee Min-Geol
Department of Dermatology, Yonsei University College of Medicine, 134 Shinchon-dong, Seodaemoon-gu, Seoul 120-752, Korea.
Yonsei Med J. 2006 Apr 30;47(2):278-81. doi: 10.3349/ymj.2006.47.2.278.
The transition between the main subtypes of pemphigus, pemphigus vulgaris (PV), and pemphigus foliaceus (PF) has rarely been reported. Moreover, the development of PV in a patient with PF is much more unusual than that of PF in a patient with PV. We report a 48-year-old man who presented with cutaneous lesions showing the typical clinical and histological features of PF. Five years later, his skin lesions became extensive and he developed oral erosions. His condition did not respond well to steroids and azathioprine. Histological examination of a vesicle disclosed suprabasal acantholysis in contrast to the subcorneal acantholysis discovered upon initial histological evaluation. Indirect immunofluorescence revealed IgG antikeratinocyte cell surface antibodies at a titer of 1:640. The titer was 1:160 at initial diagnosis. Upon immunoblotting, the patient's serum reacted with 130 kiloDalton (kDa) and 160 kDa proteins, suggesting desmoglein (Dsg) 3 and 1, respectively. We herein report an unusual case of PV that developed from PF during the disease's flare-up.
天疱疮主要亚型寻常型天疱疮(PV)和落叶型天疱疮(PF)之间的转变鲜有报道。此外,PF患者发生PV比PV患者发生PF更为罕见。我们报告一名48岁男性,其皮肤损害表现为PF典型的临床和组织学特征。5年后,他的皮肤损害范围扩大并出现口腔糜烂。他的病情对类固醇和硫唑嘌呤反应不佳。水疱的组织学检查显示基底上棘层松解,这与最初组织学评估时发现的角质层下棘层松解不同。间接免疫荧光显示IgG抗角质形成细胞表面抗体滴度为1:640。初诊时滴度为1:160。免疫印迹显示,患者血清分别与130千道尔顿(kDa)和160 kDa蛋白反应,提示分别为桥粒芯糖蛋白(Dsg)3和1。我们在此报告1例在疾病发作期间由PF发展而来的不寻常PV病例。