1/67. hyperpigmentation caused by hyperthyroidism: differences from the pigmentation of Addison's disease.Two cases of hyperthyroidism with hyperpigmentation are presented. In both cases, hyperpigmentation was seen on the lower extremities, most strikingly on the shins, backs of the feet and the nail bed. histology of the pigmented skin showed basal melanosis and heavy deposition of haemosiderin around dermal capillaries and sweat glands. Treatment with mercazol in both cases resulted in no significant waning of pigmentation. Distribution of hyperpigmentation, haemosiderin deposition and poor response to the treatment may be characteristic features of the pigmentation caused by hyperthyroidism, and may represent differences from the pigmentation seen in Addison's disease.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
2/67. Unusual association of thyroiditis, Addison's disease, ovarian failure and celiac disease in a young woman.The coexistence of autoimmune endocrine diseases, particularly autoimmune thyroid disease and celiac disease (CD), has recently been reported. We here present a 23-year-old woman with a diagnosis of hypothyroidism due to Hashimoto's thyroiditis, autoimmune Addison's disease, and kariotypically normal spontaneous premature ovarian failure. Considering the close association between autoimmune diseases and CD, we decided to search for IgA anti-endomysium antibodies (EmA) in the serum. The positivity of EmA and the presence of total villous atrophy at jejunal biopsy allowed the diagnosis of CD. On a gluten-free diet the patient showed a marked clinical improvement accompanied, over a 3-month period, by a progressive decrease in the need for thyroid and adrenal replacement therapies. After 6 months, serum EmA became negative and after 12 months a new jejunal biopsy showed complete mucosal recovery. After 18 months on gluten-free diet, the anti-thyroid antibodies titre decreased significantly, and we could discontinue thyroid substitutive therapy. This case emphasizes the association between autoimmune polyglandular disease and CD; the precocious identification of these cases is clinically relevant not only for the high risk of complications (e.g. lymphoma) inherent to untreated CD, but also because CD is one of the causes for the failure of substitute hormonal therapy in patients with autoimmune thyroid disease.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
3/67. Systemic lupus erythematosus with polyglandular autoimmune syndrome type II: report of an unusual case.A patient with systemic lupus erythematosus (SLE) is described who had associated autoimmune thyroiditis with subclinical hypothyroidism and idiopathic Addison's disease. The presence of autoimmune thyroid disease and Addison's disease could classify her as having polyglandular autoimmune syndrome (PGAS) type II, which is the commonest of the three types of this syndrome. However, the additional presence of SLE in PGAS type II has not been described earlier. This patient appears to be the first such case.- - - - - - - - - - ranking = 5keywords = gland (Clic here for more details about this article) |
4/67. Isolated pneumocystis carinii infection of adrenal glands causing Addison's disease in a non-immunocompromised adult.pneumocystis carinii is primarily an opportunistic pathogen infecting patients with AIDS and other immunocompromised patients, and ordinarily does not affect immunocompetent persons. We report isolated P. carinii infection of bilateral adrenal glands in a non-immunocompromised adult male, leading to fatal Addisonian crisis. diagnosis of P. carinii was established on the basis of cytopathology and microbiological tests, using conventional staining techniques and direct immunofluorescence on ultrasound-guided fine needle aspirates and trucut needle biopsy specimen from adrenal glands. P. carinii pneumonia and other fungal infections of the adrenal glands were excluded by appropriate tests. Absence of hiv infection was established by negative ELISA for hiv I and II antibodies and Western blot analysis at the time of presentation and 45 d later. Normal blood total leukocyte and CD4 lymphocyte counts and IgG and IgA levels confirmed the immunocompetent status of the patient. The patient improved with anti-Pneumocystis treatment and corticosteroid replacement, but succumbed to an episode of Addisonian crisis triggered by a diarrheal illness.- - - - - - - - - - ranking = 7keywords = gland (Clic here for more details about this article) |
5/67. Schmidt's syndrome in a Saudi family.We present 3 patients from a Saudi family who are presented with polyglandular autoimmune syndrome type 2. They have Addison's disease with either autoimmune thyroid disease or insulin dependent diabetic mellitus. Although this syndrome is rare, the incidence among saudi arabia or the Arab population is not known.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
6/67. A case of histoplasma capsulatum causing granulomatous liver disease and Addisonian crisis.A 56-year-old man with persistently elevated liver enzyme levels, fatigue, lethargy and a 9.0 kg weight loss over six months underwent a percutaneous liver biopsy that demonstrated multiple granulomas. Screening serologies were positive for histoplasmosis, and he was started on itraconazole treatment. He returned to hospital the same night with coffee-ground emesis and in Addisonian crisis requiring parenteral steroids and intensive care unit support. An abdominal computed tomography scan revealed bilaterally enlarged, nonenhancing adrenal glands suggestive of infarcts, presumed secondary to histoplasmosis. Treatment was initiated with amphotericin b, and histoplasma capsulatum was cultured from his urine and cerebrospinal fluid. A serum immunodiffusion test was also positive for both H and M bands, indicating active infection with histoplasmosis species. His serum and urine samples were also weakly positive for the antigen. Despite complications of renal failure, pneumonia and congestive heart failure, he recovered with medical therapy and was discharged home to complete a prolonged course of itraconazole therapy. While hepatic granulomas often reflect an occult disease process, the cause may remain undiscovered in 30% to 50% of patients despite exhaustive investigations. H capsulatum is an uncommon cause of granulomatous liver disease, and with its protean clinical presentation, a high index of suspicion is needed to make the diagnosis and avoid the potentially high fatality rate associated with disseminated infection.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
7/67. Distinct clinical phenotype and immunoreactivity in Japanese siblings with autoimmune polyglandular syndrome type 1 (APS-1) associated with compound heterozygous novel AIRE gene mutations.We herein report on two Japanese siblings with autoimmune polyglandular syndrome type 1 (APS-1). The brother, who expressed a characteristic phenotype of APS-1, had developed severe mucocutaneous candidiasis in early infancy and thereafter developed hypoparathyroidism and Addison's disease, along with a severe deterioration of his immunologic function. In contrast, the 44-year-old sister, who showed a noncharacteristic phenotype of APS-1, developed insulin-dependent diabetes with high anti-glutamic acid decarboxylase antibody, mild nail candidiasis, and autoimmune hepatitis with intact immunoreactivity. She had three susceptible human leukocyte antigen (HLA) loci for type 1 autoimmune diabetes. The expression of T cell receptor (TCR)V beta 5.1 increased in both patients, while the brother showed a widely suppressed expression of many TCRV beta families. Both individuals possessed compound heterozygous novel autoimmune regulator (AIRE) gene mutations (L29P and IVS9-1G > C). The same AIRE gene mutations can thus be associated with characteristic and noncharacteristic phenotypes of APS-1, and HLA may possibly influence the phenotype of APS-1.- - - - - - - - - - ranking = 5keywords = gland (Clic here for more details about this article) |
8/67. pituitary apoplexy presenting as Addisonian crisis after coronary artery bypass grafting.Addisonian crisis, also commonly referred to as adrenal crisis, occurs when the cortisol produced by the adrenal glands is insufficient to meet the body's acute needs. The symptoms are nonspecific and can mimic other processes, such as sepsis. hypotension, lethargy, and fever can all be presenting signs. Secondary addisonian crisis can also result from pituitary apoplexy. pituitary apoplexy usually occurs as hemorrhagic or ischemic necrosis in the presence of a pre-existing pituitary adenoma, and is a rare sequela of surgery. The symptoms of pituitary apoplexy are typically impressive and are relieved by urgent transsphenoidal decompression. hypopituitarism resulting from pituitary apoplexy can be treated with exogenous hormones. The case presented herein illustrates occult pituitary apoplexy that occurred after on-pump coronary artery bypass grafting. In this patient, the initial signs of addisonian crisis were overlooked; however, once recognized, they were reduced dramatically with standard stress-dose cortisone. A suprasellar mass with a cystic component was found on magnetic resonance imaging. The hemorrhagic pituitary gland was treated by transsphenoidal decompression, which relieved the patient's bitemporal hemianopia and 6th-nerve palsy.- - - - - - - - - - ranking = 2keywords = gland (Clic here for more details about this article) |
9/67. Adrenal lymphoma and Addison's disease: report of a case.Addison's disease due to adrenal lymphoma usually manifests as bilateral adrenal enlargement. We report a patient with Addsion's disease in whom the initial overt primary adrenal insufficiency was accompanied by an only slightly enlarged right adrenal gland. The 80-year-old man presented with nausea, anorexia, weight loss, and hyperpigmentation of the skin and buccal mucosa. Addison's disease was diagnosed based on this clinical presentation and laboratory findings of low cortisol and high adrenocorticotropin levels. Computerized tomography (CT) of the adrenal glands revealed a small right adrenal tumor. His family refused to allow percutaneous or surgical biopsy to determine the nature of the tumor. His general condition improved after steroid supplementation. However, about 1 year later, dizziness, fever, night sweats, and edema of the lower legs developed, and adrenal CT scanning revealed that the left adrenal gland had enlarged and now exceeded the size of the right one. Left adrenalectomy was performed and pathology showed a diffuse large B-cell lymphoma. Staging work-up using whole-body CT scanning suggested a stage IIIb lymphoma. Chemotherapy was given, but the disease still progressed and the patient died 4 months after diagnosis. Primary adrenal lymphoma should be considered in the differential diagnosis of Addison's disease, even if only slight enlargement of the adrenal glands is found initially.- - - - - - - - - - ranking = 4keywords = gland (Clic here for more details about this article) |
10/67. Clinical significance of adrenal computed tomography in Addison's disease.Adrenal computed tomographic (CT) scanning was conducted in twelve patients with Addison's disease during the clinical course. In tuberculous Addison's disease (n = 8), three of four patients examined during the first two years after disease onset had bilaterally enlarged adrenals, while one of four had a unilaterally enlarged one. At least one adrenal gland was enlarged after onset in all six patients examined during the first four years. Thereafter, the adrenal glands may atrophy bilaterally, in contrast to adrenal glands in idiopathic Addison's disease, which atrophy bilaterally from disease onset (n = 2). Adrenal calcification was a less sensitive clue in tracing pathogenesis, i.e., adrenal calcification was observed in five of eight patients with tuberculous Addison's disease, but not in idiopathic patients. Thus, adrenal CT scanning could show the etiology of Addison's disease (infection or autoimmunity) and the phase of Addison's disease secondary to tuberculosis, which may be clinically important for initiating antituberculous treatment.- - - - - - - - - - ranking = 3keywords = gland (Clic here for more details about this article) |
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