A distinctive vesiculopustular eruption associated with hepatobiliary disease.
Overview
abstract
BACKGROUND: Cutaneous manifestations of hepatobiliary disease may be a clue to the clinical diagnosis. We encountered a form of vesiculopustular eruption in five patients with hepatobiliary disease. A similar eruption has been described in the setting of ulcerative colitis, and has received the designation of vesiculopustular pyoderma gangrenosum. METHODS: Skin biopsy specimens were examined by conventional histopathologic methods and, in three cases, fresh tissue was available for direct immunofluorescence examination. A diagnosis of hepatobiliary disease was known in three of the patients and in the other two it was established by the skin biopsies. RESULTS: All skin biopsies of the five patients showed a neutrophilic dermatosis that had two morphologies. One was characterised by superficial neutrophilic dermolysis with subepidermal bulla formation and the other by a nonulcerating destructive suppurative folliculitis and perifollicular neutrophilic dermolysis. Both were frequently accompanied by a neutrophilic and lymphotic eccrine hidradermitis and a Sweet's syndrome-like vascular reaction. The lesions were held to represent a novel vesiculopustular eruption with features reminiscent of pyoderma gangrenosum. CONCLUSIONS: We propose the term vesiculopustular eruption of hepatobiliary disease for this distinctive clinicopathologic entity, which bears a strong histologic resemblance to pyoderma gangrenosum. Patients with a known hepatopathy who present with necrotizing vesicles and pustules may have such an eruption. The onset may be the first clue to or herald an exacerbation of underlying liver disease.