Necrolytic migratory erythema

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(Redirected from Glucagonoma syndrome)


Necrolytic migratory erythema
Synonyms N/A
Pronounce N/A
Specialty N/A
Symptoms Erythematous rash, blistering, crusting
Complications Infection, scarring
Onset
Duration
Types N/A
Causes Glucagonoma
Risks
Diagnosis Clinical diagnosis, biopsy
Differential diagnosis N/A
Prevention N/A
Treatment Surgical resection, octreotide, nutritional support
Medication N/A
Prognosis
Frequency Rare
Deaths N/A


Necrolytic migratory erythema (NME) is a distinctive skin rash that is often associated with the glucagonoma syndrome, a rare pancreatic tumor that secretes excessive amounts of the hormone glucagon.

Presentation

NME is characterized by erythematous patches that evolve into blistering and crusting lesions. These lesions typically appear in areas subject to friction or pressure, such as the groin, buttocks, perineum, and lower abdomen. The rash is often pruritic and can be painful.

Pathophysiology

The exact mechanism of NME is not fully understood, but it is believed to be related to the metabolic effects of excess glucagon, including amino acid deficiency and zinc deficiency. These deficiencies may impair the skin's ability to repair itself, leading to the characteristic lesions.

Diagnosis

Diagnosis of NME is primarily clinical, supported by the presence of a glucagonoma. A biopsy of the skin lesions can help confirm the diagnosis by showing necrolysis of the upper layers of the epidermis.

Treatment

The primary treatment for NME is the surgical removal of the glucagonoma. Medical management may include the use of octreotide, a somatostatin analog that inhibits glucagon secretion. Nutritional support, including amino acid and zinc supplementation, may also be beneficial.

Prognosis

The prognosis of NME depends on the successful treatment of the underlying glucagonoma. Surgical resection of the tumor can lead to resolution of the skin lesions and improvement in symptoms.

See also

References



External links

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Contributors: Prab R. Tumpati, MD