scholarly journals MON-912 Undiagnosed Chronic Eczema as a Presentation of Glucagonoma in MEN 1 Syndrome

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Siroj Dejhansathit ◽  
Parinya Samakkarnthai ◽  
Subhanudh Thavaraputta ◽  
Ana Marcella Rivas Mejia ◽  
Kenneth Nugent ◽  
...  
Keyword(s):  
Somatostatin Receptor ◽  
Skin Lesions ◽  
Parathyroid Glands ◽  
Pancreatic Tumors ◽  
Topical Steroids ◽  
Erythematous Plaque ◽  
Subtotal Parathyroidectomy ◽  
Normal Ranges ◽  
Necrolytic Migratory Erythema ◽  
Chronic Eczema

Abstract Background: Glucagonomas are pancreatic tumors arising from the islets cell of Langerhans that over secrete glucagon. Necrolytic migratory erythema (NME) is an important feature for the recognition of glucagonomas. Glucagonomas occurring in MEN1 is infrequent and seen in less than 3% of all glucagonomas. Clinical Case: A 51-year-old male presented to the clinic multiple visits for rash affecting the legs and genital area of two months. His medical history include type 2 DM and HT. The rash was attributed to subacute eczema and treated with topical steroids but showed no improvement. The skin eruption initially appeared on lower extremities progressed to trunk, and face. The skin lesions were associated with weight loss and stomatitis. On physical examination, skin showed ill-defined erythematous plaque exhibiting annular pattern, scale, and erosion on all extremities and perioral area. When the skin lesions healed, the new cutaneous eruptions occurred. Laboratory testing revealed plasma glucose of 185 mg/dL. The skin biopsy reported vacuolated keratinocytes in the epidermis with eosinophil cytoplasm, compatible with NME leading to further workup for pancreatic tumor. CT abdomen revealed tumor mass 9.6x6 cm at the pancreatic tail and multiple nodules in the liver. Somatostatin receptor scintigraphy showed an area of increased radiotracer uptake at the tail of the pancreas and multiple liver nodules corresponding to the previous CT scan. Serum glucagon was 923 pg/mL, confirming the diagnosis for glucagonoma. The patient was treated with distal pancreatectomy, and enucleation of liver metastases. Histopathological reported grade 2 well-differentiated NET. During the admission, the patient was found to have a parathyroid level of 79.3 pg/mL and increased uptake in the left and right lower regions of the thyroid gland from parathyroid MIBI scan, indicating hyperfunctioning parathyroid glands. All pituitary hormones were within normal ranges and no pituitary tumor was detected by the MRI brain. BMD showed osteoporosis at the lumbar spine and left femoral neck. The patient was referred to general surgery for subtotal parathyroidectomy. Pathological of resected parathyroid glands reported parathyroid hyperplasia. Postoperative PTH level and calcium were returned to normal range. Genetic testing focused on MEN1 gene, and the mutation was identified. After four months follow up plasma glucagon decreased to 425 pg/dL, patient had complete resolution of the cutaneous lesions. Conclusion: Glucagonomas are a rare pancreatic tumors and often difficult to recognize. Chronic eczema may misdiagnose for NME and delay diagnosis. NME can be challenging for physicians to recognize NME which is an important key to diagnose glucagonoma. Even though MEN1 association with glucagonoma is infrequent, awareness of such is important to allow appropriate testing for MEN1 in patients with glucagonoma.

scholarly journals Ectopic Intact PTH Secretion Causing Humoral Hypercalcemia of Malignancy

2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A191-A192
Author(s):  
Mohammad Talha Rauf ◽  
Winnie Nhan ◽  
Gregory A Clines ◽  
Shafaq Khairi
Keyword(s):  
Primary Hyperparathyroidism ◽  
Somatostatin Receptor ◽  
Liver Lesion ◽  
Receptor Expression ◽  
Parathyroid Glands ◽  
Pathological Examination ◽  
Venous Sampling ◽  
Humoral Hypercalcemia Of Malignancy ◽  
Intact Pth ◽  
Subtotal Parathyroidectomy

Abstract Background: Tumor-generated ectopic intact PTH is difficult to diagnose and should be suspected in patients with apparent primary hyperparathyroidism but with normal parathyroid glands. Clinical Case: A 72-year-old man presented with symptoms of hypercalcemia including generalized weakness, polyuria, and polydipsia. Initial labs were consistent with primary hyperparathyroidism: calcium 12.1 mg/dL (n 8.6–10.3 mg/dL, albumin-corrected 12.5 mg/dL), intact PTH (iPTH) 115.6 pg/mL (n 10–65 pg/mL), low normal 25-OH vitamin D (25 ng/mL, n 25–100 ng/mL), and relatively high normal 1,25 dihydroxyvitamin D (52 pg/mL, n 18–78 pg/mL). 24-hour urine calcium was 381 mg/day (n 100–300 mg/day) and PTHrP was 1.6 pmol/L (n <4.2 pmol/L). Neck ultrasound demonstrated a 0.5 x 1 cm hypoechoic mass near right thyroid inferior pole, though sestamibi SPECT/CT scan did not reveal scintographic evidence of a parathyroid adenoma. He underwent subtotal parathyroidectomy with largest excised gland weighing 0.262 grams. The left inferior parathyroid gland appeared normal intraoperatively, thus was clipped and left in place. PTH decreased from 194 pg/mL to 98 pg/mL postoperatively. Pathological examination revealed three normocellular parathyroid glands with enlargement of only the right superior gland. Venous sampling of the parathyroid vasculature failed to identify the source of autonomous iPTH post operatively. Due to refractory hypercalcemia, cinacalcet was initiated. However, hypercalcemia as high as 12.6 mg/dl and hyperparathyroidism to 672 pg/mL persisted despite dose escalation. He eventually received pamidronate with subsequent transition to denosumab due to declining renal function. A  68Ga DOTATATE scan was performed to locate occult ectopic parathyroid, which reported multiple foci of presumed somatostatin receptor expression involving the liver and intra-abdominal lymph nodes without significant uptake in the neck concerning for metastatic disease. Liver lesion biopsy was consistent with pancreato-biliary adenocarcinoma. Surprisingly, the biopsy was negative for iPTH and neuroendocrine tumor markers on staining/immunohistochemistry. Given his poor prognosis and multiple comorbidities, the patient opted not to pursue any further workup or therapy for his malignancy. Conclusion: Occult malignancy should be suspected for a patient with persistent hyperparathyroidism after parathyroidectomy. Treatment of the malignancy may lead to an improvement in hypercalcemia and iPTH levels. Employment of iPTH mRNA testing or intra-abdominal venous sampling to prove ectopic iPTH secretion would be ideal, as iPTH staining could be falsely negative. Further testing was not completed as the patient declined further evaluation.

scholarly journals SURGICAL ANATOMY OF PARATHYROID GLANDS IN PATIENTS WITH SECONDARY HYPERPARATHYROIDISM

2015 ◽  
Vol 7 (3) ◽  
pp. 23-28 ◽  
Author(s):  
K Yu Novokshonov ◽  
Y N Fedotov ◽  
V Y Karelin ◽  
T S Pridvizhkin ◽  
R A Chernikov ◽  
...  
Keyword(s):  
Secondary Hyperparathyroidism ◽  
Surgical Anatomy ◽  
Parathyroid Glands ◽  
Total Parathyroidectomy ◽  
Thyroid Lobe ◽  
Subtotal Parathyroidectomy ◽  
Carotid Sheath ◽  
Treat Ment ◽  
Gland Tissue ◽  
Surgical Failure

Ectopic or supernumerary parathyroid glands (PTg) can be the reason of surgical failure in treat- ment of secondary hyperparathyroidism in patients, who underwent dialysis. The aim of this study is to estimate the number and localization of PTgs in patients with secondary hyperparathyroidism. We included 165 patients, who underwent total parathyroidectomy with heterotopic autotransplantation of parathyroid gland tissue or subtotal parathyroidectomy. All identified PTgs were separated in two groups: eutopic and ectopic. Preoperative localization was performed by multispiral computed tomog- raphy of neck and mediastinum, neck ultrasonography, two-isotope Tc99 MIBI of PTgs. In postopera- tive period, we estimated the level of parathyroid hormone in the serum and performed morphological verification. There were found 659 PTgs. 12 (7,2%) patients had 3 parathyroid glands, and 11 (6.7%)had 5 PTgs. 4 Ptgs were found in 142 (86,1%) patients. 520 (78,9%) PTgs were eutopic, 139 (21,1%) - ectopic. The most common ectopic place for upper PTgs were paraesophageal and retrotracheal spaces, carotid sheath. Ectopic lower PTgs were most commonly located in the horns of the thymus. All super- numerary PTg were ectopic and often located in area between lower pole of the thyroid lobe and the thymus.Conclusion. During the operation in case when ectopy is suspected, upper PTgs should be located in in paraesophageal and paratracheal areas or in carotid sheath, if it necessary. If lower PTgs is absence, surgery should be completed cervical thymectomy.

scholarly journals Topical steroid application can induce branched/reticular vessels in Bowen disease on the upper trunk

2020 ◽  
Vol 12 (2) ◽  
Author(s):  
Hiroyo Hashimoto ◽  
Yaei Togawa ◽  
Naoki Aoyagi ◽  
Ryoji Kurita ◽  
Rena Oguma ◽  
...  
Keyword(s):  
Medical Records ◽  
Topical Steroid ◽  
Topical Steroids ◽  
Steroid Use ◽  
Keratinocyte Proliferation ◽  
History Of ◽  
Superficial Dermis ◽  
Bowen Disease ◽  
Epidermal Atrophy ◽  
Chronic Eczema

We aimed to elucidate the dermoscopic vasculature of patients with Bowen Disease (BD) that was misdiagnosed as chronic eczema and had branched and/or reticular vessels after topical steroid application. The medical records of 19 patients with BD on the upper trunk were retrospectively reviewed for steroid use history, vascular structure observed in dermoscopy, and corresponding histological findings. Four patients treated with strong topical steroids showed remarkable branched and/or reticular vessels on dermoscopy. Histopathology showed partial epidermal atrophy with irregular thin elongation of the rete ridges, atypical keratinocyte proliferation in the epidermis, and vasodilation in the superficial dermis. We considered that vasodilation and partial epidermal atrophy may be induced by topical steroid application in BD-affected areas. In cases of suspected BD with reddish-brown plaque showing branched and/or reticulated vessels in dermoscopy, confirming a history of topical steroid use is helpful.

scholarly journals Glucagonoma syndrome associated with necrolytic migratory erythema

2015 ◽  
Vol 61 (3) ◽  
pp. 203-206 ◽  
Author(s):  
Florentino de Araújo Cardoso Filho ◽  
Roney Gonçalves Fechine Feitosa ◽  
Carolina Oliveira Costa Fechine ◽  
Carlos Márcio Melo de Matos ◽  
Amanda Linhares Cardoso ◽  
...  
Keyword(s):  
Weight Loss ◽  
Chromogranin A ◽  
Pancreatic Neuroendocrine Tumor ◽  
Skin Lesions ◽  
Greater Omentum ◽  
Alpha Cells ◽  
Curative Therapy ◽  
Pancreatic Alpha Cells ◽  
Necrolytic Migratory Erythema

Summary Introduction: glucagonoma is a pancreatic neuroendocrine tumor derived from alpha-cells of the islets of Langerhans. It is marked by tumoral autonomous production of glucagon and characterized, among other symptoms, by necrolytic migratory erythema, an erythematous circinate lesion with areas of necrosis and sloughing. This is a rare disease with worldwide incidence estimated at 1 case per 20 million people. Case report: we report a case of glucagonoma associated necrolytic migratory erythema in a male patient, 56 years, with signs of skin lesions mainly on his legs and groin, hyperglycemia and weight loss. Biopsies of the skin lesions were performed and imaging of the abdomen showed a mass of 10 x 9 cm, at the pancreatic region. The patient was subjected to body-caudal pancreatectomy and splenectomy with autotransplant of the spleen in the greater omentum. The histopathologic report indicated a tumor in the pancreatic alpha cells. Immunohistochemistry showed expression of glucagon and chromogranin A in most tumor cells, consistent with the diagnosis of glucagonoma. The patient presented 3 years of outpatient follow-up with no complications. Conclusion: the necrolytic migratory erythema is important for the clinical recognition of glucagonoma, and its early diagnosis is essential for a successful curative therapy.

scholarly journals Superficial Necrolytic Dermatitis (Necrolytic Migratory Erythema) in Dogs

1993 ◽  
Vol 30 (1) ◽  
pp. 75-81 ◽  
Author(s):  
T. L. Gross ◽  
M. D. Song ◽  
P. J. Havel ◽  
P. J. Ihrke
Keyword(s):  
Hepatic Dysfunction ◽  
Serum Alkaline Phosphatase ◽  
Islet Cell Tumor ◽  
Pancreatic Tumors ◽  
Human Beings ◽  
Plasma Glucagon ◽  
Necrolytic Migratory Erythema ◽  
Hormonal Dysfunction ◽  
Hepatic Lesions ◽  
Amino Acid Concentrations

Twenty-two dogs with superficial necrolytic dermatitis were evaluated prospectively, twenty-one of which had characteristic crusting lesions of the paw pads. Histologically, epidermal lesions included parakeratosis and laminar intracellular edema. The plasma amino acid concentrations of eight dogs were markedly depressed. Nine dogs had terminal diabetes mellitus. These clinical and morphologic findings were strikingly similar to those of necrolytic migratory erythema in human beings, the most common cause of which is hyperglucagonemia due to islet cell tumor of the pancreas. No pancreatic tumors were found in these dogs; plasma glucagon concentrations in the five dogs tested were normal. The serum alkaline phosphatase concentrations were elevated in all dogs. Severe vacuolar hepatopathy, suggesting metabolically or hormonally induced hepatic dysfunction, was found in 21 dogs at necropsy or by biopsy; one dog had ultrasonographic abnormalities of the liver. Histopathologically, severe vacuolar alteration resulted in parenchymal collapse and nodular regeneration, which grossly mimicked cirrhosis. Although the definitive metabolic stimulus was not discovered for the cutaneous and hepatic lesions, the similarity of the cutaneous and biochemical features of canine superficial necrolytic dermatitis to human necrolytic migratory erythema warrants further investigation into possible underlying pancreatic hormonal dysfunction.

scholarly journals Imaging of Pancreatic Neuroendocrine Neoplasms

2021 ◽  
Vol 18 (17) ◽  
pp. 8895
Author(s):  
Giuditta Chiti ◽  
Giulia Grazzini ◽  
Diletta Cozzi ◽  
Ginevra Danti ◽  
Benedetta Matteuzzi ◽  
...  
Keyword(s):  
Functional Imaging ◽  
Imaging Techniques ◽  
Therapy Monitoring ◽  
Somatostatin Receptor ◽  
World Health ◽  
Biological Behavior ◽  
Pancreatic Tumors ◽  
Neuroendocrine Neoplasms ◽  
Imaging Features ◽  
Pancreatic Neuroendocrine Neoplasms

Pancreatic neuroendocrine neoplasms (panNENs) represent the second most common pancreatic tumors. They are a heterogeneous group of neoplasms with varying clinical expression and biological behavior, from indolent to aggressive ones. PanNENs can be functioning or non-functioning in accordance with their ability or not to produce metabolically active hormones. They are histopathologically classified according to the 2017 World Health Organization (WHO) classification system. Although the final diagnosis of neuroendocrine tumor relies on histologic examination of biopsy or surgical specimens, both morphologic and functional imaging are crucial for patient care. Morphologic imaging with ultrasonography (US), computed tomography (CT) and magnetic resonance imaging (MRI) is used for initial evaluation and staging of disease, as well as surveillance and therapy monitoring. Functional imaging techniques with somatostatin receptor scintigraphy (SRS) and positron emission tomography (PET) are used for functional and metabolic assessment that is helpful for therapy management and post-therapeutic re-staging. This article reviews the morphological and functional imaging modalities now available and the imaging features of panNENs. Finally, future imaging challenges, such as radiomics analysis, are illustrated.

scholarly journals A CASE STUDY ON MANAGEMENT OF ERYTHRODERMA

2021 ◽  
Vol 9 (5) ◽  
pp. 1131-1135
Author(s):  
Prasanna N Mogasale ◽  
Radhika. C ◽  
Nagaraj S
Keyword(s):  
Western Medicine ◽  
Skin Lesions ◽  
Topical Steroids ◽  
Exfoliative Dermatitis ◽  
History Of ◽  
Total Body ◽  
Total Remission ◽  
Therapy Treatment

Erythroderma also known as generalized Exfoliative dermatitis which refers to a scaling involving 90% or more of the cutaneous surface. Clinicians are challenged to find the cause of exfoliative dermatitis by eliciting history of illness prior to erythema. Patients presenting acutely with exfoliative dermatitis often require admission because their total body functions can require monitoring. In western medicine, the principle management is to maintain skin moisture, avoid scratching, apply topical steroids; prolonged glucocorticoids therapy often is needed. From Ayurvedic perspective it could be correlated to Eka Kusta. Eka Kusta is the Vata Kapha Pradhana Vikara. Where skin lesions are Mahavastu, Masthyashaklopamam, Krisha Aruna in Varna. Here presenting a case of 48 years old female with erythroderma who was on long- term steroidal therapy. Treatment was planned with Shamana line of management based on her Bala. The treatment is carried out for one and half month in OPD and IPD section, all the western medicine was stopped and there was total remission of symptoms with Shamana Aushadhis. Keywords: Erythroderma, Eka Kusta, Shamana Aushadhis

scholarly journals Persistent primary hyperparathyroidism: an uncommon location for an ectopic gland - Case report and review

2012 ◽  
Vol 56 (6) ◽  
pp. 393-403 ◽  
Author(s):  
Sofia Gouveia ◽  
Dírcea Rodrigues ◽  
Luísa Barros ◽  
Cristina Ribeiro ◽  
Anabela Albuquerque ◽  
...  
Keyword(s):  
Primary Hyperparathyroidism ◽  
Parathyroid Gland ◽  
Median Sternotomy ◽  
Parathyroid Glands ◽  
Aortopulmonary Window ◽  
Genetic Syndromes ◽  
Subtotal Parathyroidectomy ◽  
Common Causes ◽  
Ectopic Parathyroid ◽  
99Mtc Sestamibi

Primary hyperparathyroidism (PHPT) is a common endocrine disorder that mainly affects middle-aged women. Patients are usually asymptomatic. The disease might be ascribable to hyperplasia, carcinoma, and single or multiple adenomas. PHPT may be sporadic or familial, the latter comprising multiple endocrine neoplasia type 1 or 2A, familial benign hypocalciuria hypercalcemia, and hyperparathyroidism-jaw tumor syndrome. The most common causes for persistent PHPT are multiglandular disease, and missed abnormal ectopic or orthotopic parathyroid glands. Imaging localization studies should precede a new surgical intervention. Ectopic parathyroid glands are rarely located at the aortopulmonary window. For diagnosis confirmation, 99mTc-sestamibi SPECT/CT seems to be an advantageous test. Another possibility is to perform 99mTc-sestamibi followed by thoracic CT or MRI. Parathyroidectomy may be performed by means of median sternotomy, thoracotomy, or video-assisted thoracoscopy. We describe a case of persistent primary hyperparathyroidism due to the presence of an ectopic parathyroid gland found at the aortopulmonary window. As the investigation necessary to clarify the etiology of recurrent nephrolithiasis proceeded, the diagnosis of PHPT was determined. The patient underwent subtotal parathyroidectomy; nevertheless, PHPT persisted. Genetic syndromes that could account for this condition were excluded. Imaging studies available at that time were not able to locate abnormal glands; moreover, the patient refused to undergo surgical exploration. Later, the patient underwent 99mTc-sestamibi SPECT/CT, which revealed a parathyroid gland at the aortopulmonary window.

scholarly journals Oral lichen planus with linear lichen planus: a rare association

2019 ◽  
Vol 5 (2) ◽  
pp. 429
Author(s):  
N. Padmapriya ◽  
K. Karthikeyan
Keyword(s):  
Oral Cavity ◽  
Lichen Planus ◽  
Rare Variant ◽  
Oral Lichen Planus ◽  
Skin Lesions ◽  
Topical Steroids ◽  
Rare Association ◽  
Oral Lichen

<p class="abstract">Lichen planus (LP) is a papulosquamous disorder with both cutaneous and mucosal manifestation. Linear lichen planus is rare variant of lichen planus which occurs in the extremities. Oral lichen planus is another variant of lichen planus. Coexistence of linear lichen planus with oral lichen planus is rare and only one case has been reported before this case. A 35 year old female presented with hyperpigmented linear lesion in the leg and whitish plaques in the oral cavity. Biopsy of the skin lesions showed features of lichen planus. The patient was started on topical steroids and oral hydroxychloroquine. Patient responded to treatment.</p>

scholarly journals Successful treatment of granuloma faciale

2020 ◽  
Vol 7 (1) ◽  
pp. 129
Author(s):  
Sahar H. Alsharif ◽  
Reda H. Saifaldeen ◽  
Logain G. Alghanemi
Keyword(s):  
Successful Treatment ◽  
Topical Steroids ◽  
Erythematous Plaque ◽  
Gradual Improvement ◽  
Topical Tacrolimus ◽  
Therapeutic Modalities ◽  
Granuloma Faciale ◽  
The Face ◽  
History Of

<p class="abstract">Granuloma faciale (GF) is a chronic condition characterized by asymptomatic erythematous plaque with prominent telangiectasia presenting usually over the face. Although the condition is benign, its treatment is often unsatisfactory. Therapeutic modalities that have been tried include topical steroids and topical tacrolimus sometimes enhanced with topical dapsone. Others include intralesional corticosteroids, antimalarials, isoniazid and pulsed-dye laser. We report a case of a 58 years old female with a 1 year history of a solitary slowly progressive plaque over the nose. Diagnosis of GF was made based on the histopathological findings. The patient was started on the combination of topical tacrolimus, intralesional corticosteroids injection and oral doxycycline for 3 months. The patient showed gradual improvement in 3 months without any side effects. This case supports previous papers of successful treatment of GF with topical tacrolimus. There was no recurrence at follow-up 18 months later. It also supports the use of combination therapy especially in resistant cases.</p>

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