Cutaneous pemphigus vulgaris with skin features similar to the classic mucocutaneous type: a case report and review of the literature.
Pemphigus vulgaris (PV) is a life-threatening autoimmune blistering skin disease that specifically involves oral mucosa. It was recently shown that a very small number of patients with PV show no mucous membrane involvement although they have circulating autoantibodies directed against both desmoglein (Dsg)1 and Dsg3 that are associated with histopathological suprabasal acantholysis. These cases are classed as cutaneous-type PV. We report here a case of cutaneous-type PV that occurred in a 50-year-old man. Clinical examination revealed numerous tense and spreading blisters and erosions over the patient's entire body, similar to the classic mucocutaneous-type PV. Interestingly, none of the previously reported patients with cutaneous PV had shown skin features like those of mucocutaneous PV, whereas the present case clearly demonstrated very typical clinical features similar to those in mucocutaneous PV.
Shinkuma
S
S
Department of Dermatology, Hokkaido University Graduate School of Medicine, Sapporo, Japan. qxfjc346@ybb.ne.jp
Nishie
W
W
Shibaki
A
A
Sawamura
D
D
Ito
K
K
Tsuji-Abe
Y
Y
Natsuga
K
K
Chan
P T
PT
Amagai
M
M
Shimizu
H
H
D001323:Autoantibodies / Q000032:analysis; D019085:Fluorescent Antibody Technique, Direct; D019084:Fluorescent Antibody Technique, Indirect; D006801:Humans; D007074:Immunoglobulin G / Q000032:analysis; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008297:Male; D008875:Middle Aged; D010392:Pemphigus / Q000188:drug therapy / Q000276:immunology / Q000473:pathology*; D011239:Prednisolone / Q000627:therapeutic use; D012867:Skin / Q000276:immunology / Q000473:pathology*
D001323:Autoantibodies; D007074:Immunoglobulin G; D007166:Immunosuppressive Agents; D011239:Prednisolone