Cases reported "Addison Disease"

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1/27. 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.
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2/27. White Addison's disease: what is the possible cause?

    A case of chronic primary adrenal insufficiency without hyperpigmentation in a 64-year-old woman is reported. Due to the absence of hyperpigmentation the diagnosis was delayed and she became critically ill. During endocrine evaluation, in order to investigate the mechanism responsible for the absence of hyperpigmentation, skin biopsy was done and hormones responsible for the skin pigmentation were measured. Absence of hyperpigmentation is explained by high degree of melanosome degradation in secondary lysosomes called "compound melanosomes", which overwhelmed increased stimulation of the skin pigmentation. melanocyte-stimulating hormones were elevated with a strikingly high beta-LPH/ACTH ratio. To our knowledge, this is the first study of pathogenic mechanisms responsible for the absence of hyperpigmentation in white Addison's disease.
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3/27. Addison's disease: the potentially life-threatening tan.

    Addison's disease is a potentially life-threatening endocrine disorder. The classic dermatologic sign of Addison's disease is diffuse hyperpigmentation of the skin with focal hyperpigmentation of scars, nails, and the oral mucosa. Recognition of these dermatologic manifestations in a patient with otherwise vague constitutional symptoms warrants a consideration of Addison's disease.
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4/27. 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.
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5/27. hyperpigmentation mimicking Laugier syndrome, levodopa therapy and Addison's disease.

    The Laugier-Hunziker syndrome is an acquired, idiopathic, benign mucocutaneous hypermelanosis that usually occurs on the lips and oral mucosa, although it may appear at other sites. nails are frequently involved, mainly forming longitudinal hyperpigmented bands. We report the case of a patient that presented a typical picture of this entity, nearly 1 year after the beginning of treatment with levodopa. Two years after the first lesions occurred, she developed Addison's disease. The patient suffered from a diffuse discrete hyperpigmentation (it was more remarkable on exposed areas) and an intensification of the melanotic macules that were previously noticeable before in oral and genital mucosa, fingers, toes and nails. Hormonal replacement treatment enabled the control of laboratory and general manifestations and to decrease the degree of mucocutaneous hyperpigmentation considerably, despite initial hyperpigmented lesions persisting in described areas.
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6/27. Corticotroph cell hyperplasia in a patient with addison disease: case report.

    A woman with addison disease developed hyperpigmentation, headache, and nausea despite conventional replacement therapy with cortisone. Excessively elevated plasma adrenocorticotropic hormone (ACTH) with absence of response to administration of corticotropin-releasing factor (CRF), and roentgenological evidence of enlargement of the sella turcica, as well as detection of enlarged pituitary gland on magnetic resonance images, led to a diagnosis of ACTH-producing microadenoma, which was removed by transsphenoidal microsurgery. The specimen obtained at surgery evidenced corticotroph hyperplasia, as demonstrated by immunohistochemical staining for ACTH. Fine structure exhibited densely granulated cells with a few bundles of microfilaments and an abundance of large lysosomal bodies. Surgical removal of the hyperplasia alleviated the patient's symptoms, and hyperpigmentation faded remarkably. Her plasma ACTH level returned to normal, has remained normal for more than 3 years, and responds adequately to CRF administration.
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7/27. Unusual skin pigmentation in a patient with human immunodeficiency virus (hiv) infection.

    Diffuse addisonian hyperpigmentation in a male patient with acquired immunodeficiency syndrome related complex (ARC) is described. The etiology of pigmentation in this patient remains obscure but is most probably related to the H.I.V. infection. Other causes of addisonian hyperpigmentation are considered less likely.
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8/27. adrenoleukodystrophy initially diagnosed as idiopathic Addison's disease in two patients: the importance of early testing.

    Childhood cerebral X-linked adrenoleukodystrophy (X-ALD) is a rare neurodegenerative disease typically presenting from age 4 to 8 years in males. We report 2 cases of X-ALD in boys. The diagnosis of Addison's disease was made before the development of neurological symptoms in both cases. The first patient had hyperpigmentation of the lips and an adrenocorticotropic hormone (ACTH) level higher than 1250 pg/mL when he was 7 years 3 months old. The initial diagnosis was Addison's disease, but X-ALD was diagnosed at age 8 years 8 months, when his motor and mental function deteriorated. The second boy had hypoglycemia, skin pigmentation, and an ACTH level of 1086 pg/mL when he was 4 years 6 months old, but the diagnosis was changed from Addison's disease to X-ALD owing to deterioration in speech at age 7 years. Since both bone marrow transplantation and Lorenzo's oil are beneficial only at the early stage of disease and idiopathic Addison's disease is very rare in children, it is important to test for very-long-chain fatty acids in boys suspected of having Addison's disease.
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9/27. Diagnostic traps in porphyria: case report and literature review.

    porphyrias are metabolic disorders of heme biosynthesis, which encompass a broad range of symptoms and signs, neurologic, cutaneous or mixed. Because of lack of specificity and polymorphous clinical picture, porphyrias can mimic either neuropsychiatric, dermatologic, or gastrointestinal diseases. We present the case of a 58 years old man to whom clinical presentation suspicious of Addison's disease (melanoderma, fatigue, weight loss, intermittent abdominal pain) was the disguise of porphyria cutanea tarda. A general background of porphyrias and differential diagnosis with other forms of hepatic porphyria, as well as other causes of hyperpigmentation, are given. The clinician should be aware of the protean manifestations of porphyrias and include them in clinical judgment in various situations.
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10/27. Atypical presentation of shock from acute adrenal insufficiency in an adolescent male.

    OBJECTIVE: To report an atypical presentation of shock and acute adrenal insufficiency in an adolescent male. CASE SUMMARY: A 14-year-old boy with a history of nocturnal enuresis presented with a clinical picture suggestive of septic shock refractory to aggressive fluid and vasopressor management. history and physical examination were suggestive of shock secondary to an infectious etiology, associated with skin findings of hyperpigmentation. The laboratory studies were remarkable for normal sodium, potassium, glucose, as well as normal renin levels. hydrocortisone therapy led to improvement of his blood pressure and allowed weaning of vasopressor medications. Further laboratory studies, including adrenocorticotropic hormone stimulation test and adrenal antibodies, confirmed the diagnosis of primary adrenal insufficiency. CONCLUSION: Acute adrenal insufficiency is an uncommon cause of shock in the adolescent population. We report a clinical presentation suggestive of shock secondary to acute adrenal insufficiency remarkable for an atypical clinical and laboratory presentation. We further provide information on the management of acute adrenal crisis.
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