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A Serpiginous Eruption —Diagnosis

A Serpiginous Eruption —Diagnosis Diagnosis: Necrolytic migratory erythema with underlying glucagonoma (glucagonoma syndrome). Microscopic findings and clinical course Histopathologic examination showed acanthosis, widespread spongiosis, diffuse parakeratosis, and a superficial perivascular lymphocytic infiltrate (Figure 3). Investigations revealed a glucagon level of 1140 pg/mL (reference range 50-200 pg/mL) (to convert picograms to nanograms, multiply by 1.0). Computerized tomography (CT) of the pancreas revealed a 98 × 67-mm epigastric mass centered on the pancreatic head and neck. View LargeDownload Figure 3. The patient received somatostatin analogue injections to attempt to shrink the tumor prior to surgery. The eruption resolved within 48 hours of these injections. He later underwent a pancreaticoduodenectomy in which a well-differentiated endocrine carcinoma (consistent with a glucagonoma) was removed. A total of 12 of 33 regional lymph nodes were involved. Discussion Necrolytic migratory erythema (NME) is a rare dermatosis that is usually associated with an underlying pancreatic islet cell carcinoma with hyperglucagonaemia. This combination is known as glucagonoma syndrome (GS) and is rare, with an estimated incidence of 1 in 20 million.1 Other common manifestations include diabetes mellitus, weight loss, stomatitis, diarrhea, anemia, neuropsychiatric features, and thromboembolic tendency.2 The finding of NME was once considered pathognomonic for GS, but it can also occur without glucagonoma in conditions such as chronic liver disease, inflammatory bowel disease, heroin abuse, pancreatitis, and malabsorption syndromes.2 The typical clinical pattern of NME is a macular erythema that evolves into a serpiginous papulovesicular erosive dermatitis. NME seems to have a predilection for the perioral, perineum, lower abdominal, and flexural sites. At least 50% of patients will present with metastases.3 The exact cause of the eruption remains unknown. Normalization of glucagon levels or treatment with somatostatin analogues is reported to induce resolution of the eruption.4,5 Any deficiencies of amino acids, essential fatty acids, and zinc should be replaced because the disappearance of the eruption has been reported with their supplementation.6,7 The most specific histologic feature is superficial epithelial necrosis, but the findings are often nonspecific, and the most frequent histopathologic feature is diffuse parakeratosis.8 The cutaneous features of GS are easily recognized when presenting in a classical pattern and associated with mucosal involvement, diabetes mellitus, and weight loss. Not infrequently, however, as in our case, patients may present with atypical signs without diabetes mellitus. Early recognition may prevent metastatic disease. Return to Quiz Case. References 1. Wermers RA, Fatourechi V, Wynne AG, Kvols LK, Lloyd RV. The glucagonoma syndrome: clinical and pathologic features in 21 patients. Medicine (Baltimore). 1996;75(2):53-638606627PubMedGoogle ScholarCrossref 2. Lobo I, Carvalho A, Amaral C, Machado S, Carvalho R. Glucagonoma syndrome and necrolytic migratory erythema. Int J Dermatol. 2010;49(1):24-2920465606PubMedGoogle ScholarCrossref 3. Echenique-Elizondo M, Tuneu Valls A, Elorza Or úe JL, Martinez de Lizarduy I, Ib á ñez Aguirre J. Glucagonoma and pseudoglucagonoma syndrome. JOP. 2004;5(4):179-18515254346PubMedGoogle Scholar 4. van Beek AP, de Haas ER, van Vloten WA, Lips CJ, Roijers JF, Canninga-van Dijk MR. The glucagonoma syndrome and necrolytic migratory erythema: a clinical review. Eur J Endocrinol. 2004;151(5):531-53715538929PubMedGoogle ScholarCrossref 5. Elsborg L, Glenth øj A. Effect of somatostatin in necrolytic migratory erythema of glucagonoma. Acta Med Scand. 1985;218(2):245-2492865872PubMedGoogle ScholarCrossref 6. Sinclair SA, Reynolds NJ. Necrolytic migratory erythema and zinc deficiency. Br J Dermatol. 1997;136(5):783-7859205519PubMedGoogle ScholarCrossref 7. Alexander EK, Robinson M, Staniec M, Dluhy RG. Peripheral amino acid and fatty acid infusion for the treatment of necrolytic migratory erythema in the glucagonoma syndrome. Clin Endocrinol (Oxf). 2002;57(6):827-83112460334PubMedGoogle ScholarCrossref 8. Pujol RM, Wang C-Y, el-Azhary RA, Su WP, Gibson LE, Schroeter AL. Necrolytic migratory erythema: clinicopathologic study of 13 cases. Int J Dermatol. 2004;43(1):12-1814693015PubMedGoogle ScholarCrossref http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Dermatology American Medical Association

A Serpiginous Eruption —Diagnosis

Archives of Dermatology , Volume 148 (3) – Mar 1, 2012

A Serpiginous Eruption —Diagnosis

Abstract

Diagnosis: Necrolytic migratory erythema with underlying glucagonoma (glucagonoma syndrome). Microscopic findings and clinical course Histopathologic examination showed acanthosis, widespread spongiosis, diffuse parakeratosis, and a superficial perivascular lymphocytic infiltrate (Figure 3). Investigations revealed a glucagon level of 1140 pg/mL (reference range 50-200 pg/mL) (to convert picograms to nanograms, multiply by 1.0). Computerized tomography (CT) of the pancreas revealed a 98...
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Publisher
American Medical Association
Copyright
Copyright © 2012 American Medical Association. All Rights Reserved.
ISSN
0003-987X
eISSN
1538-3652
DOI
10.1001/archderm.148.3.385-f
Publisher site
See Article on Publisher Site

Abstract

Diagnosis: Necrolytic migratory erythema with underlying glucagonoma (glucagonoma syndrome). Microscopic findings and clinical course Histopathologic examination showed acanthosis, widespread spongiosis, diffuse parakeratosis, and a superficial perivascular lymphocytic infiltrate (Figure 3). Investigations revealed a glucagon level of 1140 pg/mL (reference range 50-200 pg/mL) (to convert picograms to nanograms, multiply by 1.0). Computerized tomography (CT) of the pancreas revealed a 98 × 67-mm epigastric mass centered on the pancreatic head and neck. View LargeDownload Figure 3. The patient received somatostatin analogue injections to attempt to shrink the tumor prior to surgery. The eruption resolved within 48 hours of these injections. He later underwent a pancreaticoduodenectomy in which a well-differentiated endocrine carcinoma (consistent with a glucagonoma) was removed. A total of 12 of 33 regional lymph nodes were involved. Discussion Necrolytic migratory erythema (NME) is a rare dermatosis that is usually associated with an underlying pancreatic islet cell carcinoma with hyperglucagonaemia. This combination is known as glucagonoma syndrome (GS) and is rare, with an estimated incidence of 1 in 20 million.1 Other common manifestations include diabetes mellitus, weight loss, stomatitis, diarrhea, anemia, neuropsychiatric features, and thromboembolic tendency.2 The finding of NME was once considered pathognomonic for GS, but it can also occur without glucagonoma in conditions such as chronic liver disease, inflammatory bowel disease, heroin abuse, pancreatitis, and malabsorption syndromes.2 The typical clinical pattern of NME is a macular erythema that evolves into a serpiginous papulovesicular erosive dermatitis. NME seems to have a predilection for the perioral, perineum, lower abdominal, and flexural sites. At least 50% of patients will present with metastases.3 The exact cause of the eruption remains unknown. Normalization of glucagon levels or treatment with somatostatin analogues is reported to induce resolution of the eruption.4,5 Any deficiencies of amino acids, essential fatty acids, and zinc should be replaced because the disappearance of the eruption has been reported with their supplementation.6,7 The most specific histologic feature is superficial epithelial necrosis, but the findings are often nonspecific, and the most frequent histopathologic feature is diffuse parakeratosis.8 The cutaneous features of GS are easily recognized when presenting in a classical pattern and associated with mucosal involvement, diabetes mellitus, and weight loss. Not infrequently, however, as in our case, patients may present with atypical signs without diabetes mellitus. Early recognition may prevent metastatic disease. Return to Quiz Case. References 1. Wermers RA, Fatourechi V, Wynne AG, Kvols LK, Lloyd RV. The glucagonoma syndrome: clinical and pathologic features in 21 patients. Medicine (Baltimore). 1996;75(2):53-638606627PubMedGoogle ScholarCrossref 2. Lobo I, Carvalho A, Amaral C, Machado S, Carvalho R. Glucagonoma syndrome and necrolytic migratory erythema. Int J Dermatol. 2010;49(1):24-2920465606PubMedGoogle ScholarCrossref 3. Echenique-Elizondo M, Tuneu Valls A, Elorza Or úe JL, Martinez de Lizarduy I, Ib á ñez Aguirre J. Glucagonoma and pseudoglucagonoma syndrome. JOP. 2004;5(4):179-18515254346PubMedGoogle Scholar 4. van Beek AP, de Haas ER, van Vloten WA, Lips CJ, Roijers JF, Canninga-van Dijk MR. The glucagonoma syndrome and necrolytic migratory erythema: a clinical review. Eur J Endocrinol. 2004;151(5):531-53715538929PubMedGoogle ScholarCrossref 5. Elsborg L, Glenth øj A. Effect of somatostatin in necrolytic migratory erythema of glucagonoma. Acta Med Scand. 1985;218(2):245-2492865872PubMedGoogle ScholarCrossref 6. Sinclair SA, Reynolds NJ. Necrolytic migratory erythema and zinc deficiency. Br J Dermatol. 1997;136(5):783-7859205519PubMedGoogle ScholarCrossref 7. Alexander EK, Robinson M, Staniec M, Dluhy RG. Peripheral amino acid and fatty acid infusion for the treatment of necrolytic migratory erythema in the glucagonoma syndrome. Clin Endocrinol (Oxf). 2002;57(6):827-83112460334PubMedGoogle ScholarCrossref 8. Pujol RM, Wang C-Y, el-Azhary RA, Su WP, Gibson LE, Schroeter AL. Necrolytic migratory erythema: clinicopathologic study of 13 cases. Int J Dermatol. 2004;43(1):12-1814693015PubMedGoogle ScholarCrossref

Journal

Archives of DermatologyAmerican Medical Association

Published: Mar 1, 2012

Keywords: exanthema,glucagonoma

References