Fox Den Disease: An Interesting Case Following Delayed Diagnosis. Wounds 2015 Jun;27(6):170-3
Date
06/11/2015Pubmed ID
26061492Scopus ID
2-s2.0-84931078950 (requires institutional sign-in at Scopus site)Abstract
Pyoderma fistulans sinifica, also known as fox den disease, is a rare and poorly understood inflammatory disorder of the skin and subcutaneous tissues. This disorder is often mistaken for other inflammatory skin disorders and treated inappropriately. The authors describe the case of a 53-year-old male who presented to the colorectal surgery service with a longstanding diagnosis of perirectal Crohn's disease. Despite aggressive immunosuppression and numerous surgical procedures, the patient continued to have unrelenting purulent drainage from the skin of his buttocks. Following wide excision of the affected skin and subcutaneous tissues by the colorectal surgeon, the plastic surgery team reconstructed the 30 cm x 55 cm wound using a combination of local flaps and skin grafts. The initial pathology report of the excised specimen confirmed the presence of nonspecific abscesses and inflammation. Upon special request by the plastic surgery team, the sample was resectioned with the specific intent of establishing a diagnosis of fox den disease. The additional slides met the criteria for an unequivocal diagnosis of fox den disease. Immunosuppression was discontinued and the patient healed his wounds without complication. Fox den disease is often overlooked because of the obscurity of the disease and the special histological sectioning needed to establish a diagnosis. In this case, the patient was unnecessarily treated with immunosuppressive drugs for more than 3 decades because of a misdiagnosis. With increased awareness of fox den disease, perhaps its pathophysiology can be better elucidated as more patients are appropriately diagnosed and treated.
Author List
Stehr RC, Kim N, LoGiudice JA, Ludwig KAuthors
John A. LoGiudice MD Professor in the Plastic Surgery department at Medical College of WisconsinKirk A. Ludwig MD Chief, Professor in the Surgery department at Medical College of Wisconsin
MESH terms used to index this publication - Major topics in bold
ButtocksContraindications
Crohn Disease
Debridement
Delayed Diagnosis
Diagnostic Errors
Graft Survival
Humans
Male
Middle Aged
Perineum
Pyoderma
Quality of Life
Rare Diseases
Recurrence
Skin Transplantation
Time Factors
Treatment Outcome