Pyodermatitis-pyostomatitis vegetans (PD-PSV) is a very rare inflammatory disease characterized by exudative pustular lesions of the skin and mucous membranes. The pathogenesis is unknown, but it may be related to immune or infectious processes. It is usually associated with inflammatory bowel disease (IBD). We describe a 32-year-old male with PD-PSV associated with manifestations suggestive of autoimmune hepatitis (AIH). To the best of our knowledge, this association has not been reported previously.
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