Necrolytic Migratory Erythema as the First Manifestation of Glucagonoma

Afsharfard, Abolfazl; Atqiaee, Khashayar; Lotfollahzadeh, Saran; Alborzi, Majid; Derakhshanfar, A

doi:10.1155/2012/974210

Cited by 6 publications

(5 citation statements)

References 9 publications

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“…The perineum, distal limbs, lower abdomen and face are the most commonly affected sites. 2 NME is an obligatory paraneoplastic syndrome, usually associated with pancreatic neuroendocrine tumours (PNETs). Glucagonomas are considered among the rarest of the PNETs, which produce a well-defined clinical syndrome characterized by NME, Type 2 diabetes mellitus (T2DM), glossitis, anaemia and weight loss.…”

Section: Discussionmentioning

confidence: 99%

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Thick crusted plaques on the periorificial area in a young woman

Pathania

Budania

Mudugal

et al. 2022

Clinical and Experimental Dermatology

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A 22-year-old woman presented with a 1-year history of multiple itchy, oozy, brown to black, crusted plaques over the face, trunk and limbs. The patient was thin, and she had also been experiencing recurrent stomatitis and reduced appetite within that time, with weight loss of > 10 kg. There was no history of any systemic problem. Mucocutaneous examination revealed multiple, well-defined, variably sized, charcoal-black to brownblack coloured crusted plaques with perilesional erythema, predominantly over the perioral and malar area (Figs. 1 and 2) and in the flexures of the arms and legs (Supplementary Fig. S1a,b).

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Section: Discussionmentioning

confidence: 99%

“…The erythematous scaly lesions with a centrifugal growth pattern characterize the clinical appearance of the disease. The perineum, distal limbs, lower abdomen and face are the most commonly affected sites 2 …”

Section: Discussionmentioning

confidence: 99%

Thick crusted plaques on the periorificial area in a young woman

Pathania

Budania

Mudugal

et al. 2022

Clinical and Experimental Dermatology

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“…There are many reports of delayed diagnosis of glucagonoma due to misdiagnosis or delayed diagnosis of skin lesions. [9][10][11][12][13][14] The average time from recognized symptoms to diagnosis is about 4 years. 15 Histology may show nonspecific dermatitis, requiring multiple biopsies to confirm the diagnosis.…”

Section: Discussionmentioning

confidence: 99%

Case of delayed diagnosis of necrolytic migratory erythema

Abdelli

Alaoui

Souissi

et al. 2021

Clinical Case Reports

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Necrolytic migratory erythema (NME) is a rare cutaneous paraneoplastic manifestation of glucagonoma. Estimated incidence of glucagonoma is 1/20,000,000/year. 1 Elevated glucagon level can cause a variety of clinical manifestations including weight loss, diabetes, and NME. This skin rash has a cyclic nature, and lesions exist simultaneously at different stages. 2 Misdiagnosis of paraneoplastic cutaneous manifestation could delay the diagnosis of glucagonoma. We report an interesting case of a female patient with a 6-year history of delayed diagnosis of glucagonoma. | CASE REPORTA 36-year-old Tunisian woman had been followed in our dermatology department for 6 years. She was referred to us for chronic prurigo. Laboratory testing revealed hypochromic microcytic anemia. Serum glucose, HBA1C, and liver plasma tests were normal. She had normal serum zinc, albumin, and lipid levels. Abdominal ultrasound was normal. She was treated with topical steroid intermittently with variable response. Since this episode, she had returned several times with polymorphous skin lesions such as papules, erythematosquamous and crusty plaques, vesicles, pustules, and erosions accompanied by severe pruritus. At that time, we thought she had prurigo (Figure 1A), eczema (Figure 1B), insect bites (Figure 1C), or even drug eruption (Figure 1D).The eruption was episodic with spontaneous exacerbations and remissions. The patient had no abdominal pain, gastrointestinal symptoms, or weight loss. Multiple skin biopsies were performed. They concluded to drug eruption, eczema, prurigo, and erythema multiforme. During the course of the outbreaks, the patient developed angular cheilitis and gingivitis, a deep vein thrombosis in the leg, and psychological problems which affected her social and professional life. Six years later, she presented with acute abdominal and pelvic pain. Abdominal computed tomography (CT) was performed, and hyperdense mass was confirmed on body-tail pancreatic of 15 cm in maximum diameter with mild degree of contrast enhancement. Removal of the tumor was indicated and the cutaneous lesions vanished 1 week after surgery. Pathology report indicated a tumor in the pancreatic alfa

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“…The polymorphism of the cutaneous features and the rarety of glucagonoma delayed the recognition of the clinical syndrome. There are many reports of delayed diagnosis of glucagonoma due to misdiagnosis or delayed diagnosis of skin lesions (6)(7)(8)(9)(10)(11). The average time from recognized symptoms to diagnosis is about four years (12).…”

Section: Discussionmentioning

confidence: 99%