Necrolytic migratory erythema

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Necrolytic migratory erythema

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Necrolytic migratory erythema (NME) is a classical symptom observed in patients with glucagonoma and is present in 80% of cases. Associated NME is characterized by the spread of erythematous blisters and swelling across areas subject to greater friction and pressure, including the lower abdomen, buttocks, perineum, and groin.

Diagnosis

Symptoms

It consists of serpiginous (slow progressing) erythematous plaques. Where the migratory edge has an "eroded" appearance. It usually starts in the Perineum.

Associated symptoms

Weight loss, anemia , mild diabetes, diarrhea and glossitis are associated. Liver metastasis is often present.

Erythema Characteristics

• Migratory circinate erythema/plaques with areas of necrosis and sloughing

• Crusted Erythematous scaly plaques with centrifugal growth
• Associated Conditions
• Obligatory paraneoplasticsyndrome
• First manifestation of the rare pancreatic neuroendocrine tumor(glaucagonoma)
• No other association
  • Can be misdiagnosed as:
    • Contact dermatitis
    • Intertrigo
    • Inverse psoriasis
    • Zinc deficiency
    • Other nutritional deficiencies

Histopathology

• Paleness and spongiosis of the upper layer of the epidermis

• A perivascularlymphocytic and histiocytic infiltrate

• Necrotickeratinocytes are common and can lead to erosions, crusting and scaling

Lab finding & Other evaluation

• Increased glucagonlevel

• Evaluation of the associated tumor:
  • CT or MRIabdomen

• * Selective visceral angiographyto localize the tumor

• * Positron Emission Tomography(PET)

• * Octreotidescintigraphy

prognosis

• Due to the difficulty of necrolytic migratory erythema recognition, and its association with glucagonoma, diagnosis is usually delayed
  • Necrolytic migratory erythema usually resolved after the resection and treatment of the pancreatic tumor,eg.10 days after tumor resection
  • Early recognition is crucial for better diagnosis and prognosis Differential Diagnosis:
• Erythema gyratum repens (EGR)
• Erythema annulare centrifugum (EAC)
  

References

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