Multicentric Castleman's disease associated with inherited epidermolysis bullosa.
Multicentric Castleman's disease (MCD) is a rare disorder characterized by fever, polyclonal hypergammaglobulinemia, and generalized lymphadenopathy. It has three histological characteristics: a recognizable architecture, germinal center abnormalities, and plasmacytosis. Inherited epidermolysis bullosa (EB) is also a rare disorder caused by a genetic defect. We report a 43-year-old patient with dystrophic EB, non-Hallopeau-Siemens recessive type or dominant type, displaying clinicopathologic features of MCD. In addition, his serum interleukin-6, which is thought to be responsible for the clinical symptoms in MCD, was elevated.
Kawakami
Yoshio
Y
Department of Dermatology, Fukushima Medical University School of Medicine, Fukushima, Japan.
Nishibu
Akiko
A
Kikuchi
Satoshi
S
Ohtsuka
Mikio
M
Nakamura
Koichiro
K
Nozawa
Yoshihiro
Y
Abe
Masafumi
M
Iwatsuki
Keiji
K
Kaneko
Fumio
F
D000328:Adult; D044466:Asian People / Q000235:genetics; D005871:Castleman Disease / Q000097:blood / Q000150:complications / Q000175:diagnosis* / Q000235:genetics / Q000473:pathology; D003937:Diagnosis, Differential; D004820:Epidermolysis Bullosa / Q000097:blood / Q000150:complications / Q000175:diagnosis* / Q000235:genetics / Q000473:pathology; D020022:Genetic Predisposition to Disease; D006801:Humans; D015850:Interleukin-6 / Q000097:blood; D007564:Japan; D008297:Male
D015850:Interleukin-6