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CASE REPORT
Year : 2015  |  Volume : 4  |  Issue : 3  |  Page : 188-191

Pseudoglucagonoma syndrome secondary to pancreatitis: A case report


Department of Dermatology Venereology and Leprology, Kurnool Medical College, Kurnool, Andhra Pradesh, India

Date of Web Publication15-Sep-2015

Correspondence Address:
Yerrajwala Aruna Kumari
Flat No. 203, A Block, SV Pride, Birla Guest House Compound, Kurnool, Andhra Pradesh
India
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Source of Support: Nil., Conflict of Interest: There are no conflicts of interest.


DOI: 10.4103/2277-8632.165396

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  Abstract 

Necrolytic migratory erythema (NME) is pathognomonic of glucagonoma syndrome associated with pancreatic neoplasm. Pseudoglucagonoma syndrome, which is extremely rare, refers to NME in the absence of glucagon-secreting tumor. We describe a case of NME in a 22-year-old female who presented with skin rash with scaling, cheilitis, glossitis associated with hair loss, and loss of weight. Serum glucagon, amino acids, and zinc levels were normal. Ultrasonography (USG) and computed tomography (CT) abdomen showed chronic pancreatitis. Resolution of lesions was observed with topical steroids, emollients, and intravenous (IV) protein infusions and other supplements.

Keywords: Chronic pancreatitis, necrolytic migratory erythema (NME), pseudoglucagonoma syndrome


How to cite this article:
Kumari YA, Reddy IC, Sree KG, Ala M. Pseudoglucagonoma syndrome secondary to pancreatitis: A case report. J NTR Univ Health Sci 2015;4:188-91

How to cite this URL:
Kumari YA, Reddy IC, Sree KG, Ala M. Pseudoglucagonoma syndrome secondary to pancreatitis: A case report. J NTR Univ Health Sci [serial online] 2015 [cited 2020 Mar 28];4:188-91. Available from: http://www.jdrntruhs.org/text.asp?2015/4/3/188/165396


  Introduction Top


Glucagonoma syndrome, first described by Becker et al. in 1942,[1] is characterized by pancreatic islet cell tumor associated with necrolytic migratory erythema (NME), diabetes mellitus, hyperglucagonoma, hypoaminoaciduria, cheilitis, anemia, loss of weight, venous thrombosis, and psychiatric disturbances. NME associated with conditions other than glucagonoma refers to pseudoglucagonoma syndrome. It is extremely rare, with a prevalence of 1 in 20,000,000. The causes include pancreatitis, gastrointestinal disorders, inflammatory bowel disease, and hepatic cirrhosis.

We report a case of NME in a female patient with chronic pancreatitis.


  Case Report Top


A 22-year-old female presented with intermittent skin rash for 1 year's duration. Initially the rash started over the inner thighs and genitalia and gradually involved the perineum, lower limbs, upper limbs, and back. The rash relapsed and remitted spontaneously. There was history of mild itching associated with rash, loss of appetite, loss of weight, and diffuse hair loss. There was a history of pain abdomen and diarrhea on and off for the past 2 years. She was diagnosed as a case of abdominal tuberculosis (TB) and was given anti-TB therapy. She had taken topical emollients as well as protein and iron supplements as prescribed by a local doctor and found some relief, but there was recurrence. On general examination, she was found to be moderately built and ill nourished, and her vitals and other systems were normal.

On cutaneous examination, widespread, circumscribed, bilateral and symmetrical erythematous and eroded plaques with scaling were present predominantly over the upper and lower limbs, genitalia, and back. Irregular erythematous plaques with peripheral crusting were present over the back. Angular cheilitis and stomatitis were present. The tongue was beefy red in color and painful. There was diffuse noncicatricial alopecia with brownish scalp hair, eyebrows, and eyelashes [Figure 1], [Figure 2], [Figure 3], [Figure 4].
Figure 1: Bilaterally symmetrical erythematous and eroded plaques over back

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Figure 2: Irregular erythematous plaques with peripheral crusting over back of neck

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Figure 3: Bilaterally symmetrical erythematous plaques over lower limbs

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Figure 4: Angular cheilitis with beefy red tongue

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Clinically, NME was suspected and the case was evaluated.

Routine investigations showed normocytic normochromic anemia and raised liver enzymes. Erythrocyte sedimentation rate (ESR), random blood sugar (RBS), renal function test (RFT), serum electrolytes, serum glucagon, and serum amino acids were within normal limits. Skin biopsy showed moderately dense, superficial, perivascular mixed infiltrate of lymphocytes and neutrophils with mild ballooning of the upper epidermis. The keratinocytes in the upper dermis showed pink staining of their cytoplasm and darkly stained small nuclei suggestive of pyknosis. The diagnosis was consistent with NME [Figure 5].
Figure 5: Moderately dense superficial perivascular mixed infiltrate of lymphocytes and neutrophils with mild ballooning of upper epidermis. The keratinocytes in upper epidermis show pink staining of their cytoplasm and darkly stained small nuclei suggestive of pyknosis. Diagnosis is consistent with NME

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The chest x-ray was normal, while ultrasonography (USG) abdomen showed calcification and atrophy in pancreas. Upper gastrointestinal (GI) endoscopy and colonoscopy were normal. Computed tomography (CT) scan abdomen showed chronic pancreatitis with dilated pancreatic duct with multiple pancreatic calculi, and gall bladder calculi. CT chest revealed calcified hilar lymph nodes [Figure 6].
Figure 6: CT scan abdomen: Chronic pancreatitis with dilated pancreatic duct, with multiple pancreatic calculi and gall bladder calculi

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Based on clinical, histopathological, and radiological findings, a diagnosis of pseudoglucagonoma syndrome secondary to chronic pancreatitis was considered. The patient was treated with topical corticosteroids, emollients, and intravenous (IV) protein infusions, zinc, vitamin B complex, and iron supplements. The lesions regressed with moderate improvement of dermatitis [Figure 7] and [Figure 8].
Figure 7: Resolution of rash following nutrition supplementation

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Figure 8: Resolution of glossitis following treatment

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The patient was followed up for 6 months, and there was no recurrence of skin lesions. She was on maintenance with emollients and oral supplements.


  Discussion Top


Pseudoglucagonoma syndrome refers to NME in the absence of glucagon-secreting tumor that characterizes glucagonoma syndrome. This may occur in pancreatitis, gastrointestinal disorders such as inflammatory bowel disease, nonpancreatic malignancies, iatrogenic causes, and odontogenic abscess.[2]

NME presents as waves of irregular erythema, with central bullae followed by erosions and crusting of advancing edge, glossitis, cheilitis, and stomatitis, associated with glucagonoma or liver/GI disorders.[3] Confluent, annular, pruritic, and painful plaques, typically waxing and waning, developed in areas of pressure and friction, starting in the perineum, groin, and then the buttocks, lower abdomen, and lower limbs.[4]

NME usually occurs in the context of glucagonoma with very high serum levels of glucagon. However, NME with normal glucagon levels has been reported in celiac sprue and pancreatitis. This may be mediated by enteroglucagon, a substance that is produced by the crypt cells of the small intestine in the malabsorptive state. Unabsorbed nutrients in the lumen are potent stimulators of enteroglucagon, which can cause NME by an undetermined mechanism.[5]

A thorough literature search revealed only a few cases of NME without glucagonoma. NME is reported in a heroin-dependent patient,[6] one iatrogenic case,[7] and the another associated with zinc deficiency.[8]

Recently in a retrospective review, only two cases were reported with acute pancreatitis who were alcohol-dependent.[9] Cutaneous eruptions, which may go undiagnosed for years before a correct diagnosis is made, are not infrequent.[9]

Our case showed normal biochemical values and multiple pancreatic calculi on CT, which was pathognomonic of chronic pancreatitis. Kawa et al. found that pancreatic stones developed more frequently in one-third of patients with autoimmune pancreatitis who presented as clinically symptomatic or asymptomatic with structural pancreatic changes and autoantibodies.

NME associated with glucagonomas has good prognosis with rapid resolution of skin rash following surgical resection of tumor.

Pseudoglucagonoma syndrome has a protracted course as the factors underlying it, such as pancreatitis, are almost irreversible, and the treatment should be directed toward the underlying condition. Supplementation with essential amino acids (EAA), zinc, and essential fatty acids (EFA) improved NME in some patients.[10]

NME is a unique clinical sign that should arouse suspicion of glucagonomas. Hence our case raises the necessity of thorough evaluation for other conditions such as pancreatitis and liver and GI disorders apart from glucagonoma.

Acknowledgment

We give sincere thanks to Dr. Uday Khopkar, dermatopathologist, Mumbai.

 
  References Top

1.
Becker SW, Kahn D, Rothman S. Cutaneous manifestations of internal malignant tumors. Arch Dermatol Syphilol 1942;45:1069-80.  Back to cited text no. 1
    
2.
Tierney EP, Badger J. Etiology and pathogenesis of necrolytic migratory erythema: Review of the literature. Med Gen Med 2004;6:4.  Back to cited text no. 2
    
3.
Espana A. Figurate erythemas. In: Bolognia JL, Jorizzo JL, Schaffer JV, editors. Dermatology. 3rd ed. Philadelphia: Saunders; 2012. p. 310.  Back to cited text no. 3
    
4.
Bhat R, Khaitan BK. Skin manifestations in internal diseases. In: IADVL Text book of Dermatology. 3rd ed. Mumbai: Bhalani Medical Publisher; 2013. p. 1391.  Back to cited text no. 4
    
5.
Bencini PL, Vigo GP, Caputo R. Necrolytic migratory erythema without glucagonoma in a heroin-dependent patient. Dermatology 1994;189:72-4.  Back to cited text no. 5
    
6.
Mullans EA, Cohen PR. Iatrogenic necrolytic migratoty erythema: A case report and review of nonglucagonoma-associated necrolytic migratory erythema. J Am Acad Dermatol 1998;38:866-73.  Back to cited text no. 6
    
7.
Sinclair SA, Reynolds NJ. Necrolytic migratory erythema and zinc deficiency. Br J Dermatol 1997;136:783-5.  Back to cited text no. 7
    
8.
Echenique-Elizondo M, Tuneu Valls A, Elorza Orúe JL, Martinez de Lizarduy I, Ibáñez Aguirre J. Glucagonoma and pseudoglucagonoma syndrome. JOP 2004;5:179-85.  Back to cited text no. 8
    
9.
Echenique-Elizondo M, Elorza JL, De Delas JS. Migratory necrolytic erythema and glucagonoma. Surgery 2003;133:449-50.  Back to cited text no. 9
    
10.
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;57:827-31.  Back to cited text no. 10
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8]



 

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