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

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Presentation on theme: "Case 31 Clinical Details supplied:  72 year old female  Erosive plaques natal cleft and groin."— Presentation transcript:

1 Case 31 Clinical 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.

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6 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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10 Histological features.  Acanthosis.  Spongiosis.  Vacuolation of upper dermal keratinocytes.  Apoptotic keratinocytes.  Parakeratin.  Neutrophils in upper dermis.  IMF negative.

11 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.

12 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.

13 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.

14 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  January 2009 developed Type II diabetes.  May 2009 – CT abdomen progression of liver mets with thickening of small bowel wall. ? Involvement but no obstruction.

15 ?Glucagonoma syndrome. Necrolytic migratory erythema.

16 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.

17 Necrolytic migratory erythema  Pseudoglucagonoma syndrome

18 Thank you


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