Glucagonoma Syndrome
Introduction
Introduction to glucagonoma syndrome Glucagonoma syndrome (glucagonomasyndrome), also known as necrolysis of migratory erythema (necrolyticmigratoryerythema) is characterized by repeated necrotic loosening erythema, stomatitis, weight loss, diabetes, and often accompanied In the syndrome of pancreatic cell tumors that secrete glucagon, about 80% of the tumors are malignant, and half of them may have metastasis. Skin lesions often appear before the tumor for several years, and the cause is still unclear. It is speculated that it is related to hypersecretion of glucagon. basic knowledge The proportion of illness: 0.002% Susceptible people: no special people Mode of infection: non-infectious Complications: venous thrombosis, diarrhea, mental disorder
Cause
Cause of glucagonoma syndrome
Cause:
The cause is not clear, and it is speculated that it is related to hypersecretion of glucagon.
Pathogenesis:
The pathogenesis is still not very clear.
Prevention
Prevention of glucagonoma syndrome
There is no effective preventive measure for this disease. Early detection and early diagnosis are the key to the prevention and treatment of this disease.
Complication
Complications of glucagon syndrome Complications, venous thrombosis, diarrhea, mental disorder
And often associated with the secretion of glucagon only pancreatic cell tumor syndrome, about 80% of the tumor is malignant, half may have metastasis.
Symptom
Symptoms of glucagonoma syndrome Common symptoms Blood sugar increased stomatitis Diabetes hypoproteinemia Tongue hypokalemia
It is a syndrome of pancreatic cell tumors characterized by recurrent necrolysis of migratory erythema, stomatitis, weight loss, and diabetes, often accompanied by secretion of glucagon. About 80% of tumors are malignant, and half of them are malignant. There is metastasis, skin lesions often appear before the tumor for several years, especially in middle-aged, especially menopausal women, skin lesions can be generalized to the body friction and vulnerable to traumatic parts, trauma, compression and friction can induce and aggravate the disease, skin At the beginning of the damage, the size is different, polygon or irregular reddish plaque, occasionally miliary to mung bean big pimples and herpes, it becomes dark purple red within 1 to 2 days, the center is pale or purple, and then the central blisters appear. Or pustules, easy to rupture to form erosion and crusting, desquamation left a pale brown to bronze pigmentation, erythema edge spread to the periphery, the clear red crusting edge of the boundary is ring-shaped or multi-ring, most patients have serious Glossy tongue, scarlet, stripped with mild atrophy, looks like beef, consciously stinging, more than half of patients have mild to moderate diabetes, intermittent diarrhea, bloating, vomiting and melena, patients with weak body, Reduced weight, can hypokalemia, anemia, mental disorders, and venous thrombosis.
Examine
Examination of glucagonoma syndrome
ESR increased, anemia, hypoproteinemia, intermittent diabetes, elevated fasting blood glucose, abnormal glucose tolerance test, elevated blood glucagon levels greater than 5000mg / L can be diagnosed.
Histopathological examination: the lesions at the edge of the lesions with early lesions were characterized by biopsy, epithelial layer of superficial layer of intracellular edema, cytoplasmic eosinophilic, nuclear pyknosis, necrosis, leading to fissures and blister formation, necrosis Neutrophils can be seen in the area, sometimes forming a sponge-like abscess, there is no granular layer, 2/3 of the lower part of the spinous cell layer is normal, dermal papillary edema, vasodilation, and mild lymphocytic infiltration around the perivascular.
Diagnosis
Diagnosis and diagnosis of glucagonoma syndrome
Diagnostic points:
1. More common in women, especially in middle-aged, especially menopausal women, skin lesions can be generalized, but it occurs in the lower abdomen, groin, perineum, genitals, buttocks, thighs, mouth and nose, extremities, Friction and vulnerability to traumatic sites, trauma, compression and friction can induce and aggravate the disease.
2. The lesions are initially of different sizes, polygonal or irregularly shaped reddish erythema, occasionally miliary to mung bean big papules and herpes, which become dark purple red within 1 to 2 days, center pale or purplish-like, then appear in the center Superficial blister or pustule, easy to rupture to form erosion and crusting, desquamation left pale brown to bronze pigmentation, erythema edge spread to the periphery, clear red crusting edge of the boundary is ring-shaped or multi-ring, from erythema After 7 to 14 days of pigmentation, the rash is one after another, the damage can be resolved by itself, periodically after several weeks to several months, the folds are smashed, the erythema of the mouth and nose is crusted, and the fingertips are reddish brown with scaling. Some patients have scaly rash or papules, similar to eczema-like dermatitis.
3. Most patients have severe glossitis, which is scarlet, stripped with mild atrophy, looks like beef, consciously stinging, also sees angular cheilitis, conjunctivitis, vaginitis, etc., nails become thin, brittle, hair Sparse.
4. More than half of the patients have mild to moderate diabetes, intermittent diarrhea, bloating, vomiting and melena, patients with weak body, weight loss, hypokalemia, anemia, venous thrombosis and mental disorders.
5. Laboratory examination of erythrocyte sedimentation rate, anemia, hypoproteinemia, intermittent diabetes, elevated fasting blood glucose, abnormal glucose tolerance test, blood levels of glucagon increased more than 5000mg / L can be diagnosed, medical system examination, if necessary A laparotomy can confirm the tumor.
6. Histopathology takes early lesions on the edge of the lesion for biopsy with characteristic lesions, epithelial layer of superficial layer of intracellular edema, cytoplasmic eosinophilic, nuclear pyknosis, necrosis, leading to fissures and blister formation, necrosis Neutrophils can be seen in the area, sometimes forming a sponge-like abscess, there is no granular layer, 2/3 of the lower part of the spinous cell layer is normal, dermal papillary edema, vasodilation, and mild lymphocytic infiltration around the perivascular.
Need to be associated with toxic epidermal necrolysis, chronic familial benign pemphigus, subarachnoid pustulosis, pustular psoriasis, enteropathic acral dermatitis, cutaneous candidiasis, eccentric annular erythema and Severe seborrheic dermatitis identification.
