Pemphigus vulgaris presenting in a radiation portal.
We report a case of mucocutaneous pemphigus vulgaris in a patient with squamous cell carcinoma of the lung. The cutaneous involvement was limited to the skin within his therapeutic radiation portal. The diagnosis of pemphigus vulgaris was confirmed by histopathology and immunologic studies. Direct immunofluorescence demonstrated IgG and C3 in the intercellular spaces and indirect immunofluorescence was positive on monkey esophagus at a titer of 1:160. Enzyme-linked immunosorbent assay of the patient's serum detected autoantibodies only to desmoglein (Dsg)3, with no reactivity to Dsg1. Immunomapping of perilesional skin from the irradiated field illustrated decreased Dsg1 expression compared with a control sample from an area that was not exposed to radiation. This case provides support for the Dsg compensation hypothesis and may also suggest a mechanism by which irradiation may induce skin lesions.
Robbins
Amber C
AC
Department of Dermatology, Medical College of Wisconsin, Milwaukee, USA.
Lazarova
Zelmira
Z
Janson
Marleen M
MM
Fairley
Janet A
JA
D001323:Autoantibodies / Q000097:blood / Q000378:metabolism; D002294:Carcinoma, Squamous Cell / Q000532:radiotherapy; D003176:Complement C3 / Q000378:metabolism; D051183:Desmoglein 1 / Q000378:metabolism; D051301:Desmoglein 2 / Q000276:immunology; D004797:Enzyme-Linked Immunosorbent Assay; D004817:Epidermis / Q000276:immunology / Q000378:metabolism; D005110:Extracellular Space / Q000378:metabolism; D019085:Fluorescent Antibody Technique, Direct; D019084:Fluorescent Antibody Technique, Indirect; D006801:Humans; D007074:Immunoglobulin G / Q000378:metabolism; D008175:Lung Neoplasms / Q000532:radiotherapy; D008297:Male; D008875:Middle Aged; D010392:Pemphigus / Q000209:etiology* / Q000276:immunology / Q000378:metabolism / Q000473:pathology; D011832:Radiation Injuries / Q000150:complications*; D012867:Skin / Q000276:immunology / Q000378:metabolism
D001323:Autoantibodies; D003176:Complement C3; C495297:DSG1 protein, human; C495314:DSG2 protein, human; D051183:Desmoglein 1; D051301:Desmoglein 2; D007074:Immunoglobulin G