Q J Med 2002; 95: 834-835
© 2002 Association of Physicians
Correspondence |
Glucagonoma syndrome presenting as psoriasis
1 Department of Dermatology, 2 Department of Pathology, 3 Department of Radiology, 4 Department of Endocrinology, 5 Department of Biochemical Medicine and 6 Department of Surgery, Ninewells Hospital, Dundee e-mail: paula_e_beattie{at}hotmail.com
Sir,
The cutaneous eruption of glucagonoma is described as a distinct clinical entity, but in the early stages of this disease, clinical signs may be subtle. We present a patient who presented with psoriasis and subsequently was diagnosed as having glucagonoma syndrome.
A 40-year-old man presented in June 1998 with a year history of an erythematous scaling seborrhoeic dermatosis, diagnosed clinically and histologically as psoriasis, and a 3 month history of an intermittent sore/cracked tongue, but no other relevant history.
Over the next two years, his rash relapsed and remitted, with elements of impetigo at times, and latterly the condition developed into an acral and seborrheoic papulosquamous dermatosis (Figure 1
), with lower leg erosions and a papulo-pustular rash on the face. He also had two episodes of self-limiting vomiting, dehydration and weight loss (13 kg at one point in time), developed a deep venous thrombosis and had intermittent lethargy, recurrent mouth ulceration and balanitis. He was extensively investigated and found to have mild intermittent lymphopenia, oesophageal candidiasis on endoscopy, and glycosuria on one occasion. Negative or unremarkable investigations included biochemistry (including serum glucose), protein electrophoresis, immunoglobulins, HIV status, hepatitis serology, lymphocyte subsets and function tests, abdominal ultrasound scan, barium meal and follow-through, (a previous) endoscopy, oesophageal and gastric biopsies, and a pancreolauryl test.
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In February 2001 a repeat skin biopsy from the eroded areas on the leg demonstrated widespread hydropic degeneration and clefting of the stratum corneum, with some neutrophils in the upper epidermis and abscess formation. A differential diagnosis of acrodermatitis enteropathica, pellagra and glucagonoma was considered. Serum zinc levels were within reference intervals, and zinc supplementation did not produce benefit. Investigation of gut hormones and neuroendocrine markers demonstrated massively elevated glucagon levels (8000 pg/ml, normal <250 pg/ml), and raised fasting insulin (23 mU/l, normal <15), pancreatic polypeptide (3240 ng/l, <200), gastrin (120 ng/l, <100) and chromogranin A (148 U/l, 2–20). Levels of VIP and glucose were normal, as were calcium and prolactin. MRI of the abdomen demonstrated a large tumour in the body and tail of the pancreas, with no metastases (Figure 2
). He underwent a distal pancreatectomy and splenectomy with subsequent rapid resolution of all symptoms and hormone profiles.
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Psoriasis affects 1–2% of the population, with a variety of clinical patterns. Drug eruptions, mycosis fungoides, HIV, hepatitis C infection, nicotinic acid or zinc deficiency, and glucagonoma syndrome can mimic psoriasis.
Glucagonomas are 
-cell tumours of the pancreas with an incidence of 1:20 million.1 Approximately 60% are malignant, and the associated paraneoplastic condition, glucagonoma syndrome, is present in approximately 57%.2 Features include glucose intolerance or diabetes, painful glossitis, stomatitis or cheilitis, perineal lesions, anaemia, weight loss, diarrhoea, venous thrombosis, psychiatric disturbance and necrolytic migratory erythema (NME), one of the commonest and often earliest-presenting signs.
Skin lesions exhibit a cyclical pattern, appear as macules, develop central bullae then erode and heal leaving hyperpigmentation and a crusted periphery. Centrifugal extension produces a serpiginous pattern. It is frequently atypical and misdiagnosed.
Rarely, NME can occur in association with malabsorption, liver disease, pancreatitis, and non-glucagon-secreting tumours3 and so cannot solely be attributed to hyperglucagonaemia, with deficiencies of amino acids,1 fatty acids or zinc4 also being postulated.
Treatment includes surgical resection, leading to prompt resolution of the rash, although intravenous amino-acids and somatostatin/octreotide may be necessary for symptom palliation.
Bone-marrow suppression by glucagon5 is thought to explain the anaemia and possibly this patient's lymphopenia. In retrospect, his DVT, glossitis and balanitis were pointers to the diagnosis, but typical features such as diabetes and diarrhoea were absent.
The variable appearance of the rash, subtle clinical signs and rarity of the syndrome, often lead to long delays in diagnosis, by which time up to 90% have metastases.1 Early recognition permits prompt surgical resection, which may be curative. And although psoriasis is extremely common, patients with unusual or unresponsive ‘psoriasis' should have other conditions considered.
References
1. Mallinson CN, Bloom SR, Warin AP, Salmon PR, Cox B. A glucagonoma syndrome. Lancet1974; 2:1–5.[Medline]
2. Soga J, Yakuwa Y. Glucagonomas/diabetico-dermatogenic syndrome (DDS): a statistical evaluation of 407 reported cases. J Hepatobiliary Pancreat Surg1998; 5:312–19.[Medline]
3. Mullans EA, Cohen PR. Iatrogenic necrolytic migratory erythema: a case report and review of nonglucagonoma-associated necrolytic migratory erythema. J Am Acad Dermatol1998; 38:866–73.[Medline]
4. Blackford S, Wright S, Roberts DL. Necrolytic migratory erythema without glucagonoma: the role of dietary essential fatty acids. Br J Dermatol1991; 125:460–2.[Medline]
5. Frankton S, Bloom SR. Gastrointestinal Endocrine tumours. Glucagonomas. Baillière's Clin Gastroenterol1996; 10:697–705.[Medline]
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