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SELF ASSESSMENT ANSWERS an Interesting Case of Thirst and Polyuria a Proliferating Pimple

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SELF ASSESSMENT ANSWERS an Interesting Case of Thirst and Polyuria a Proliferating Pimple Postgrad Med J 2002;78:251–254 251 Postgrad Med J: first published as 10.1136/pmj.78.918.254 on 1 April 2002. Downloaded from SELF ASSESSMENT ANSWERS A proliferating pimple Q1: What is the differential diagnosis 99m on clinical examination? An interesting case of thirst uptake on Tc thyroid uptake scan (fig 1; see p 248) and positive TSHRAb. The possible differential diagnosis on clinical and polyuria TSHRAb binds to the thyroid stimulating examination is: Q1: What are the possible causes of hormone receptor, activates adenylate cyclase, • Dermatofibroma. thirst and polyuria in this woman? and increases thyroid hormone production • Pyogenic granuloma. The two likely causes of thirst and polyuria in and secretion. The TSHRAb in Graves’ disease • Malignant melanoma. this patient are central diabetes insipidus sec- is referred to as stimulating or agonist type • Metastatic oat cell carcinoma. ondary to previous pituitary surgery and TSHRAb, while other varieties including a • Angiosarcoma. hypercalcaemia. In our patient, a normal blocking TSHRAb may also be present. The water deprivation test excluded central blocking antibody may be coincident with the Q2: What does the histology reveal diabetes insipidus. stimulating antibody. TSHRAb are not detect- (see p 249) and what histological Cranial surgery accounts for 20% cases of able in the normal population. A total of 80% techniques are used to establish the central diabetes insipidus in adults. After to 100% of untreated hyperthyroid patients diagnosis? pituitary surgery, persistent polyuria develops with Graves’ disease have detectable TSHRAb only when the injury is sufficiently high in the with thyroid stimulating activity.1 The histology reveals malignant round cells supraopticohypophyseal tract to cause degen- 99mTechnetium pertechnate, like iodine, is consistent with a Merkel cell carcinoma or eration of the supraoptic and paraventricular actively concentrated by the thyroid gland, trabecular carcinoma. This is a rare primary nucleus. Thus although transient diabetes undergoes negligible organic binding, and cutaneous neuroendocrine tumour arising insipidus may follow any injury to the neuro- diffuses out of the thyroid as the plasma con- from Merkel cells. hypophysis, permanent cranial diabetes insip- centration decreases. The short half life (six History 1 idus is uncommon after pituitary surgery. hours) and consequent low radiation makes it The Merkel cell is a round cell in the basal Hypercalcaemia causes transient nephro- suitable for thyroid imaging. Thyroid scinti- layer of the epidermis named after Fredrick genic diabetes insipidus manifested primarily graphy is used in the evaluation of nodular Sigmund Merkel who discovered it in 1875. as a defect in maximum renal concentrating thyroid disease, thyroid cancer, ectopic thyroid These round cells may be isolated or arranged ability accompanied by a reduction in tissue, and thyroiditis. It is not recommended in clusters around hair follicles, which are glomerular filtration rate. Other factors may in the evaluation of straightforward Graves’ innervated and function as touch receptors. include reduction in medullary solute content The tumour was first described by Toker in and inhibition by calcium of adenylate cyclase disease. In our patient, a diffusely increased 1972 who, along with Tang in 1978, described activation by arginine vasopressin in hormone uptake (even though thyroxine replacement sensitive epithelia.1 was stopped only seven days earlier) con- the intracytoplasmic dense core granules firmed the diagnosis of autoimmune hyper- similar to those seen in neuroendocrine cells.1 Q2: What are the possible causes of thyroidism. Q3: Discuss the clinical features and hypercalcaemia? management of this lesion The possible causes of hypercalcaemia include Q4: Is there an association between Clinical features thyrotoxicosis, relative glucocorticoid insuffi- acromegaly and thyrotoxicosis? ciency during illness and stress, dehydration, The Merkel cell carcinoma is a rare but acromegaly, and hyperparathyroidism as part Acromegaly is well known to be associated aggressive tumour presenting as a dark red to of multiple endocrine neoplasia syndromes. with goitre as part of generalised organo- dark blue papule. It usually arises on the head, Total serum calcium is raised in 27% and megaly. In a series of 80 patients with neck, and the extremities of the elderly.2 It is ionised calcium raised in up to 47% hyperthy- acromegaly from an iodine deficient region, often associated with other neoplasms, most roid patients.2 Patients have low PTH and 71% had goitre compared with 35% in commonly with a squamous cell carcinoma 1,25-dihydroxycholecalciferol concentrations, patients with prolactinomas. Goitres were previously resected from the same anatomical 1 and hypercalciuria. Serum calcium concen- more common in female acromegalic patients site. The two may also coexist. It is also asso- http://pmj.bmj.com/ trations are generally less than 2.7 mmol/l, but compared with males. Hyperthyroidism was ciated with Bowen’s disease and basal cell life threatening hypercalcaemia has been noted in 5% cases and none were due to carcinoma. The most important differential described. Thyroid hormones absorb bone Graves’ disease.6 Graves’ disease in patients diagnosis is metastatic oat cell carcinoma as directly, although indirect resorption may also with active or previous acromegaly has been there are no specific histological differentiat- occur by activation of local factors such as reported very rarely in the literature and pre- ing features between the two. It is, therefore, interleukin-1 or by increasing sensitivity to sented an unusual diagnostic challenge in this important to undertake a careful history and 3 β circulating PTH. -Adrenergic blocking case, specially as she was on thyroxine examination followed by chest radiography to 1 agents can reverse the hypercalcaemia as can replacement therapy after hypopituitarism. rule out a primary lung lesion. The tumour definitive therapy with carbimazole.3 has a high incidence of local recurrence (39%) on September 27, 2021 by guest. Protected copyright. Hypercalcaemia is detected in 6% cases of Final diagnosis and regional recurrence (46%). Distant me- primary adrenal insufficiency but it is less tastasis occur to bones, liver, and lung. common in secondary hypoadrenalism. Thyrotoxicosis due to Graves’ disease causing Raised calcium binding proteins caused by hypercalcaemia in a patient with hypopitui- Histology haemoconcentration are a factor, but volume tarism after treatment of acromegaly. The cellular pattern ranges from trabecular to repletion with saline does not restore calcium an insular or to a diffuse growth but all may concentrations to normal, for which glucocor- References coexist in a single tumour. The lesion tends to ticoid replacement is required.1 occupy the whole of the dermis with sparing Wilson JD Growth hormone stimulation of renal 1α- 1 , Foster WD, Kronenberg HM, et of epidermis by a thin zone of Grenz. al, eds. Williams textbook of endocrinology. Cytologically the cells are monomorphic with hydroxylase activity increases serum 1,25- 9th Ed. Philadelphia: W B Saunders, 1998. dihydroxycholecalciferol concentrations re- 2 Diamond T, Vine J, Smart R, et al. Thyrotoxic scanty cytoplasm and homogenous nuclei sulting in increased intestinal calcium bone disease in women: a potentially with a central nucleolus (arrowed fig 1A). absorption and hypercalciuria. Hypercalcae- reversible disorder. Ann Intern Med They may show a moulding effect as seen in mia is uncommon in the absence of associated 1994;120:8–11. small cell carcinomas. 1 hyperparathyroidism and has been reported 3 Pont A. Unusual causes of hypercalcemia. Staining techniques in none4 to 10%5 of cases in various series of Endocrinol Metab Clin North Am Silver stains are negative, indicating the small patients with acromegaly. 1989;18:753–64. In this patient the hypercalcaemia was due 4 Pumarino H, Oviedo S, Michelsen H, et al. number of neurosecretory granules. to thyrotoxicosis and glucocorticoid defi- [Active acromegaly and gigantism: some Immunohistochemistry ciency. It responded partially to rehydration clinical characteristics of 50 patients.] Rev This is helpful in differentiating Merkel cell with isotonic saline and intravenous hydro- Med Chil 1991;119:897–907. Ezzat S carcinoma from other tumours such as cortisone and normalised completely after 5 , Melmed S, Endres D, et al. Biochemical assessment of bone formation lymphoma, melanoma, and metastatic oat cell hyperthyroidism was treated with pro- and resorption in acromegaly. J Clin carcinoma. pranolol and carbimazole. Endocrinol Metab 1993;76:1452–7. The useful markers studies are: Wuster C Q3: What is the cause of her 6 , Steger G, Schmelzle A, et al. • Positive for low molecular weight keratins Increased incidence of euthyroid and thyrotoxicosis? hyperthyroid goiters independently of such as AE1 or CAM 5.2, epithelial mem- Her thyrotoxicosis is due to Graves’ disease as thyrotropin in patients with acromegaly. Horm brane antigen, neuron specific enolase (fig proved by homogenously increased tracer Metab Res 1991;23:131–4. 1B), and chromagranin.3 www.postgradmedj.com 252 Self assessment answers Discussion Postgrad Med J: first published as 10.1136/pmj.78.918.254 on 1 April 2002. Downloaded from Erythema gyratum repens is one of several paraneoplastic eruptions which, while rare, are important for signifying the presence of internal malignancy. Such rashes often pre- cede
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