title: Pyoderma Gangrenosum-A New Manifestation of Wilson Disease?
abstract: Seventeen year old girl, a known case of Wilson disease presented to us with a non-healing skin ulcer followed by appearance of jaundice, ascites and progressive fatigue of 1 month duration. She was diagnosed to have Wilson disease 5 years back and had been well controlled on d-penicillamine. On enquiry, she was found to be noncompliant with her medication in the preceding 6 months. On examination, she had severe pallor, icterus with moderate ascites and oedema feet. Investigations revealed severe haemolytic anemia and deranged liver function. The lesion was diagnosed to be pyoderma gangrenosum on skin biopsy. The appearance of a cutaneous lesion followed by deterioration in the liver disease and hemolysis suggested uncontrolled Wilson disease as the triggering factor. Chelation therapy improved her haemoglobin and liver function as well as led to healing of the ulcer. We describe pyoderma gangrenosum as a new manifestation of Wilson disease.
authors.affiliation: Visiting Fellow, Department of Gastroenterology, Jaslok Hospital and Research Centre, Mumbai, India.
authors.forename: George Naiem Ibrahiem
authors.initials: GN
authors.lastname: Freg
authors.affiliation: Department of Dermatology, Jaslok Hospital and Research Centre, Mumbai, India.
authors.forename: Venisha
authors.initials: V
authors.lastname: Shah
authors.affiliation: Department of Gastroenterology, Jaslok Hospital and Research Centre, Mumbai, India.
authors.forename: Aabha
authors.initials: A
authors.lastname: Nagral
authors.affiliation: Department of Gastroenterology, Jaslok Hospital and Research Centre, Mumbai, India.
authors.forename: Ajay
authors.initials: A
authors.lastname: Jhaveri
keywords: ALP, alkaline phosphatase; ALT, alanine transaminase; AST, aspartate transaminase; Ds-DNA, double stranded-deoxyribo nucleic acid; K–F, ringsKayser–Fleischer rings; WBCs, white blood cells; d-penicillamine; extra hepatic; liver; skin