Acute onset of necrolytic migratory erythema mimicking an erythema multiforme

Glucagonoma syndrome consists of the tumour of pancreatic alpha cells and symptoms caused by the abnormal glucagon production. A hallmark sign of this syndrome is necrolytic migratory erythema (NME) first described in 1942 [1]. Its distinctive clinical appearance along with concomitant diabetes mellitus, weight loss, deep vein thrombosis, neuropsychiatric disorders and diarrhoea may suggest the diagnosis. Treatment is curative if the early diagnosis is made and involves tumour surgical resection [2, 3].
A 49-year-old woman was admitted to the Dermatology Department with a skin rash possibly preceded by drug intake (acetylsalicylic acid, ibuprofen) due to the upper respiratory tract infection. Herpes simplex viral (HSV) infection history was negative. On physical examination she presented diffuse round erythematous macules, some of them creating targetoid lesions, over the neck (Figure 1 B), trunk (Figure 1 C), thighs and elbows along with acral symmetric cellulitis and painful blisters on the dorsal and palmar surfaces (Figure 1 A). Paronychia of almost all fingers was observed. There were small erosions in oral mucosa. The hair and nails were not affected. The patient was obese (BMI 30.5 kg/m2) and diagnosed with mild diabetes 4 months earlier. The family history was negative for dermatological diseases and unremarkable for other conditions. Laboratory investigations revealed lymphocytosis 51.5% (20–40%), hyperglycaemia 6.34 mmol/l (3.9–5.5) and erythrocyte sedimentation rate (ESR) 48 mm/h. Other tests including biochemistry, renal and hepatic profile, thyroid function tests, IgE, ASO were normal. Hepatitis B virus (HBV) and HCV serology were negative. Imaging examinations (chest X-ray and abdominal ultrasonography) were within normal limits. Histopathological examination could be consistent with drug reaction (Figure 2 A). She was diagnosed with erythema multiforme and treated with steroids (dexamethasone intramuscular followed by methylprednisolone per os) and clarithromycin with good response.
After 2 months the patient was readmitted to our Department due to the worsening of the skin condition. The physical examination revealed erythematous papules and crusted patches predominantly involving the intertriginous regions and scattered over the trunk (Figures 3 A–C). The lesions spread outward leaving postinflammatory hyperpigmentation. The patient reported pruritus and persistent angular cheilitis. Laboratory tests showed an elevated...


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