Discussion
Glucagonoma syndrome (GS) is a rare paraneoplastic phenomenon. Its most
common features are weight loss, NME and diabetes mellitus (3).NME is
considered a hallmark clinical sign of glucagonoma syndrome, present in
approximately 70% of patients (3).The dermatosis evolves in 7–14 days,
occurring in spontaneous exacerbations and remissions(4). Cutaneous
features can mimic bullous dermatitis; it may also present as
psoriasiform or eczematous plaques(5). Our patient’s cutaneous
lesion were under recognized and we misinterpreted the skin
manifestations as more common entities such as prurigo, eczema, and drug
eruption for six years. 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(6–11). The average time from recognized symptoms
to diagnosis is about four years (12). Histology may show non specific
dermatitis, requiring multiple biopsies to confirm the diagnosis. The
most specific feature includes superficial epithelial necrosis of the
upper spinous layer with vacuolated keratinocytes. Lack of specific
findings on biopsy and the rarity of the pancreatic tumor can attribute
to delays in diagnosis(4).
NME is a valuable cutaneous presentation in this diseases, when the skin
lesions relapsed, we were convinced of the recurrence of the tumour even
though the scan was normal.
By the time of diagnosis, 50-100% of patients already present with
metastatic disease, and a cure is often impossible(2). However,
since this islet cell tumor is slow-growing, prolonged survival (more
than 20 years) is possible, and in metastatic disease, most causes of
death appear to be unrelated to the tumor(13). Causes of death are
correlated to thromboembolism, sepsis, and gastrointestinal bleeding
(14).Skin changes appear early in the course of GS and are reason for
seeking medical help for the first time. They are followed by systemic
symptoms such as weight loss, diarrhea, diabetes mellitus,
neuropsychiatric disorders, anemia, and thrombosis.
Diabetes mellitus is found in 80% of patients with the GS (3). Our
patient had no diabetes and this delayed the recognition of the clinical
syndrome.
Glucagonoma syndrome is associated with a high incidence of
thromboembolism, estimated between 10 and 30% of patients (15) which is
responsible for the immediate cause of death in up to 50% of patients
(16). Our patient was diagnosed with deep vein thrombosis in the leg.
Complete resection of the tumor is the best treatment. Our patient’s
cutaneous lesions vanished one week after surgery. Patients who
underwent resection had longer median survival than patients who did not
receive surgery, even when diagnosed with later stages of
disease(17).