Varicella-zoster virus (VZV) and alpha 1 antitrypsin: a fatal outcome in a patient affected by endemic pemphigus foliaceus.
BACKGROUND
Herpes virus infections are well known infectious complications of pemphigus and bullous pemphigoid. We describe pathologic findings utilizing autopsy tissue from several organs from a patient affected by a new variant of endemic pemphigus in El Bagre, Colombia, South America.


METHODS
We describe a patient by a new variant of endemic pemphigus foliaceus from El Bagre that was receiving high-dosage immunosuppressants when hospitalized and died suddenly following contact with a second patient affected by chicken pox.


METHODS
We performed studies utilizing hematoxylin and eosin, immunohistochemistry, and direct immunofluorescence techniques on tissues from several organs.


RESULTS
We detected the presence of varicella zoster virus, as well as strong positivity for α-1 antitrypsin in the heart, kidneys, spleen, liver, skin, brain, lungs, pancreas, small and large intestines, and skeletal muscle. In regard to structural damage in the kidney and heart, we believe the observed damage is associated with the presence of autoantibodies to these organs, since both of them are rich in plakins and El Bagre-EPF patients present significant antibodies to plakin molecules.


CONCLUSIONS
In patients with endemic pemphigus foliaceus, we recommend complete isolation of the patient when receiving high dosages of systemic immunosuppressive agents. We further suggest the clinical possibility of a synergistic, fatal interaction between active pemphigus foliaceus, varicella zoster virus, herpes simplex virus, immunosuppressive agents, and a systemic activation of α-1 antitrypsin. Thus, we suggest adequate bed spacing, barrier nursing, and preventative testing for α-1 antitrypsin activation are warranted in these patients to address these complications.
Georgia Dermatopathology Associates, Atlanta, GA, USA. abreuvelez@yahoo.com
Ana Maria
AM
Abreu Velez
Bruce R
BR
Smoller
Weiqing
W
Gao
Hans E
HE
Grossniklaus
Zhe
Z
Jiao
Luis F
LF
Arias
Samuel C
SC
Dudley
Michael S
MS
Howard
D000212:Acyclovir / Q000627:therapeutic use; D000328:Adult; D000998:Antiviral Agents / Q000627:therapeutic use; D001379:Azathioprine / Q000627:therapeutic use; D002644:Chickenpox / Q000150:complications* / Q000188:drug therapy / Q000378:metabolism; D019353:Endemic Diseases; D017809:Fatal Outcome; D014645:Herpesvirus 3, Human; D006801:Humans; D007166:Immunosuppressive Agents / Q000009:adverse effects / Q000627:therapeutic use*; D008297:Male; D010392:Pemphigus / Q000150:complications* / Q000188:drug therapy* / Q000201:enzymology; D011241:Prednisone / Q000627:therapeutic use; D000515:alpha 1-Antitrypsin / Q000378:metabolism*
D000998:Antiviral Agents; D007166:Immunosuppressive Agents; D000515:alpha 1-Antitrypsin; D001379:Azathioprine; D011241:Prednisone; D000212:Acyclovir