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Case 31 Clinical Details supplied:

Case 31 Clinical Details supplied:. 72 year old female Erosive plaques natal cleft and groin. 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.

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Case 31 Clinical Details supplied:

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  1. Case 31Clinical Details supplied: • 72 year old female • Erosive plaques natal cleft and groin

  2. 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.

  3. 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

  4. Histological features. • Acanthosis. • Spongiosis. • Vacuolation of upper dermal keratinocytes. • Apoptotic keratinocytes. • Parakeratin. • Neutrophils in upper dermis. • IMF negative.

  5. 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.

  6. 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.

  7. 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.

  8. 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.

  9. Necrolytic migratory erythema. ?Glucagonoma syndrome.

  10. 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.

  11. Necrolytic migratory erythema • Pseudoglucagonoma syndrome

  12. Thank you

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