Post on 31-Jan-2022
transcript
PSEUDO GLUCAGONOMA SYNDROMESapna Raghunathan MD1, Annie Heenan MD2, Padmaja Akkireddy MD1
1Department of Diabetes, Endocrinology and Metabolism, 2Division of Internal Medicine, University of Nebraska Medical Center, Omaha, NE 68198
png
• Biopsy of rash showed: Confluent parakeratosis and mild
spongiosis (NME vs Nutritional Deficiency
• Tierney EP, Badger J. Etiology and pathogenesis of necrolytic
migratory erythema: review of the literature. MedGenMed 2004
• Necrotizing migratory erythema (NME) is hallmark
clinical finding of Glucagonoma, an islet cell tumor of
the pancreas.
• NME can sometimes be seen in the absence of
Glucagonoma, a condition referred to as Pseudo-
glucagonoma syndrome(PGS).
• We report a case of NME associated with severe
nutritional deficiency.
CASE• 48 y/o female presented to dermatology clinic with
diffuse itchy rash of 6 months duration.
• Rash started on the arms and spread to involve lower
abdomen, legs and perioral area. Unresponsive to both
topical and high dose PO steroids.
• Biopsy showed confluent parakeratosis and mild
spongiosis secondary to nutritional deficiencies vs
NME.
• Medical history was significant for Nissen
fundoplication 20 years ago with revision to Roux-en Y
gastric by-pass, 6 years ago. Non compliant with post-
bariatric care and vitamin supplementation.
• Admitted to the hospital for sepsis from secondary
infection of the rash.
• She was started on enteral feeds with nutritional
supplementation with significant improvement of her
rash.
PATHOLOGY
PHYSICAL EXAM
DISCUSSION• NME in the absence of Glucagonoma is extremely rare
and is seen in hepatic cirrhosis, malabsorption
disorders, inflammatory bowel disease and nutritional
deficiencies including zinc deficiency, Pellagra,
Kwashiorkor.
• The exact mechanism for NME in these conditions is
unclear. It is postulated that unabsorbed nutrients in the
gut lumen are potent stimulators of enteroglucagon
which in turn mediates the development of NME.
• Treatment of NME associated with PGS is to correct
the underlying cause.
• Our patient had history of gastric bypass surgery and
did not get routine post bariatric care. She presented
with multiple nutritional deficiencies which likely caused
NME. It is important to recognize that post bariatric
surgery, patients are at risk for both macro and
micronutrient deficiencies and hence need frequent
nutrition assessment, supplementation and monitoring.
REFERENCES
BACKGROUND LABS
LAB VALUE NORMAL VALUES
Vitamin B12 1,026 180-914 pg/mL
Vitamin A 289 300-1000 mcg/L
Vitamin E 4.1 6.5-16.5 mg/L
Vitamin B2 184 5-50 nmol/L
Vitamin B6 10.7 20-125 nmol/L
Selenium 64 23-190 µg/L
Zinc 38 60-120 µg/L
Glucagon 15 8-57 pg/mL
HbA1c 5.8 4.0-6.0 %