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Case 31 Clinical Details supplied: 72 year old female Erosive plaques natal cleft and groin
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Clinical features Spring 2009 developed an erosive intertriginous rash. Painful. Background of stasis eczema/eczema craquale on lower legs for 2 years. Waxing and waning - also painful. Treated for episodes of cellulitis.
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History Rash lower legs – eczema. New erosive rash intertriginous areas. Punch biopsy of affected area natal cleft. Punch biopsy of normal skin for IMF – negative. ? Paraneoplastic pemphigus ? Pemphigus
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Histological features. Acanthosis. Spongiosis. Vacuolation of upper dermal keratinocytes. Apoptotic keratinocytes. Parakeratin. Neutrophils in upper dermis. IMF negative.
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Diagnosis. Necrolytic migratory erythema. Glucagonoma syndrome. Pseudoglucagonoma syndrome. Zinc deficiency. Niacin ( Vit B3) deficiency – Pellagra. Due to abnormal liver function and impared glucagon metabolism. Malabsorbtion. Acrodermatits enteropathica. Necrolytic acral erythema.
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Glucagonoma syndrome. Rare – incidence of 1:20 million. Glucagon producing pancreatic islet cell tumour. Serum glucagon levels – reference lab. Slowly progressive. Hyperglucagonaemia, DM, glossitis, anaemia, nausea, diarrhoea, abdo pain, neuro symptoms, thromboembolic symptoms and weight loss.
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Other relevant history. Hypothyroidism – partial thyroidectomy for follicular adenoma. Raynauds. Several TIA’s. Weight loss. Congenital absence of gall bladder. Splenectomy and partial pancreatectomy in 1996 for pancreatic neuroendocrine tumour.
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Pancreatic neuroendocrine tumour. Liver metastases noted at the time. No history of rash at time of initial diagnosis. Rx with interferon before rash developed. Stable as of April 2008. January 2009 developed Type II diabetes. May 2009 – CT abdomen progression of liver mets with thickening of small bowel wall. ? Involvement but no obstruction.
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?Glucagonoma syndrome. Necrolytic migratory erythema.
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Unanswered questions. Exact nature of tumour? Secreting glucagon? Carcinoid syndrome can lead to Pellagra. Time line. No history of rash at presentation. Recent diagnosis of DM. Serum glucagon levels? Zinc levels – low end of normal spectrum. Response to dermovate.
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Necrolytic migratory erythema Pseudoglucagonoma syndrome
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Thank you
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