C M Magro1, A N Crowson. 1. Department of Pathology, Beth Israel Hospital, Harvard Medical School Pathology Services, Inc., Massachusetts, USA.
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.
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.
Authors: Cynthia Magro; A Neil Crowson; Linda Franks; Panta Rouhani Schaffer; Patrick Whelan; Gerard Nuovo Journal: Clin Dermatol Date: 2021-07-25 Impact factor: 3.541