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221. Porphyria cutanea tarda

hyperpigmentation, and dark or reddish urine. Factors that contribute to susceptibility include alcohol use, smoking, hepatitis C, HIV, iron overload, hereditary haemochromatosis gene mutations, oestrogen use, and uroporphyrinogen decarboxylase (UROD) mutations. Results from an acquired, substantial deficiency of UROD in the liver. Diagnosis is established by finding substantial increases in porphyrins in urine or plasma. Treatment includes repeated phlebotomy or low-dose hydroxychloroquine or chloroquine (...) (Chapter 58). In: Kaushansky K, Lichtman MA, Beutler E, et al, eds. Williams Hematology, 9th ed. New York, NY: McGraw-Hill;2016: 889-914. PCT is usually associated with liver cell damage. History and exam presence of risk factors blistering skin lesions skin hyperpigmentation hypertrichosis scarring alopecia red urine males, middle age, white alcohol use smoking oestrogen use hepatitis C HIV hereditary haemochromatosis gene (HFE) mutation uroporphyrinogen decarboxylase (UROD) mutations exposure

2018 BMJ Best Practice

222. Assessment of hypocalcaemia

examination may show dermatitis, eczema, hyperpigmentation, psoriasis, brittle hair with patchy alopecia, and brittle nails with characteristic transverse grooves. [Figure caption and citation for the preceding image starts]: Nail dystrophy due to hypocalcaemia Nijjer S et al. BMJ Case Reports 2010;2010:bcr.08.2009.2216 [Citation ends]. Patients rarely may have congestive heart failure, angina, and hypotension. Differentials Iatrogenic postsurgical hypoparathyroidism Vitamin D deficiency Hypomagnesaemia

2018 BMJ Best Practice

223. Bullous pemphigoid

or urticarial lesions for weeks or months. In the bullous stage, characteristic, tense vesicles or bullae develop on apparently normal or erythematous skin of the pre-existing eczematous or urticarial eruption. If the blisters burst, the eroded, crusty areas slowly heal to leave post-inflammatory hyperpigmentation. The lesions are usually symmetrical and favour the flexural aspects of the extremities, lower trunk, and abdomen. The treatment goal is to decrease or stop blister formation, to promote healing

2018 BMJ Best Practice

224. Overview of dermatitis

clearly delineated with sharp borders. Hyperpigmentation, fissuring, and scaling may also occur. Belsito DV. The diagnostic evaluation, treatment, and prevention of allergic contact dermatitis in the new millennium. J Allergy Clin Immunol. 2000 Mar;105(3):409-20. After an allergen or irritant is identified, the main goals of treatment are avoidance of future exposure and resolution of existing dermatitis. ICD is treated with exposure

2018 BMJ Best Practice

225. Addison's disease

Addison's disease Addison's disease - Symptoms, diagnosis and treatment | BMJ Best Practice You'll need a subscription to access all of BMJ Best Practice Search  Addison's disease Last reviewed: February 2019 Last updated: December 2018 Summary Presentation may be acute or insidious, with substantial fatigue and weakness associated with mucocutaneous hyperpigmentation, hypotension and/or postural hypotension, and salt craving. Adrenocorticotrophic hormone stimulation test is performed (...) in children is not specifically covered in this topic, but should result in referral to a paediatric endocrinology specialist. History and exam presence of risk factors fatigue anorexia weight loss hyperpigmentation salt craving nausea vomiting hypotension arthralgia and myalgia female sex adrenocortical autoantibodies adrenal haemorrhage autoimmune diseases tuberculosis (TB) non-TB bacterial infection fungal infection HIV drugs that inhibit cortisol production metastatic malignancy coeliac disease

2018 BMJ Best Practice

226. Acne

, affecting approximately 80% of people at some point between (...) 11–30 years of age. Complications of acne include scarring, hyperpigmentation, and psychological problems. In general, it is recommended that people with acne : Do not wash more than twice a day. Use a mild soap or cleanser and lukewarm water. Do not use vigorous scrubbing when washing acne -affected skin, and the use of abrasive soaps, cleansing granules, astringents, or exfoliating agents should be discouraged. Should not attempt

2018 Trip Latest and Greatest

227. Hereditary Hemochromatosis

radiographically by its specific involvement of the second and third metacarpophalangeal joints and the hook-shaped osteophyte of the metacarpal head ( ). | Skin Hyperpigmentation may be one of the earlier signs of HH ( ). Iron deposits in the skin lead to increased melanin production and deposition, giving rise to the characteristic metallic or slate gray hue commonly referred to as bronzing ( ). Hypermelanotic skin pigmentation is usually generalized but frequently is deeper on the face, neck, extensor

2019 American College of Gastroenterology

228. Elmiron (pentosan polysulfate sodium): rare risk of pigmentary maculopathy

retrospective study, patients with pigmentary maculopathy had a median length of exposure to pentosan polysulfate of 18.3 years with a range of 3.0–21.9 years. Unique characteristics of pigmentary maculopathy associated with Elmiron The pigmentary maculopathy described differs from other forms. Fundus examination showed unique subtle paracentral hyperpigmentation at the level of the retinal pigment epithelium (RPE) with associated areas of RPE atrophy. Multi-modal retinal imaging demonstrated abnormalities

2019 MHRA Drug Safety Update

230. Emergency management of adrenal insufficiency in children: advocating for treatment options in outpatient and field settings

, acute onset of nausea and vomiting for the previous 24 hours. Physical examina- tion revealed an ill-appearing, thin male with tachycardia (pulse 110 bpm), mild hypoten- sion (85/60 mm Hg), signs of dehydration, and hyperpigmentation. Laboratory testing showed hyponatremia (sodium 129 mEq/L), hyperka- lemia (potassium 5.8 mEq/L) and hypoglycemia (glucose 55 mg/dL). Despite urgent fluid resus- citation with 2 intravenous boluses of normal saline and a bolus of 10% dextrose, hypoten- sion persisted (...) to gain weight and hypoglycemia may be the presenting clinical features in neonates and infants. Micropenis, bilateral cryptorchidism and, rarely, central diabetes insipidus may also be present in neonates who have AI due to panhypopituitarism. Individ- uals with primary AI may have hyperpigmentation of the skin (particularly creases, folds and scars), gums and buccal mucosa. general biochemistry In acute AI, hyponatremia is the most consistent biochem- ical finding. 12 Hyperkalemia is present

2019 Pediatric Endocrine Society

234. Hereditary Cancer Syndromes and Risk Assessment

with Cowden syndrome-associated neoplasms and condi- tions, which are classified as pathognomonic, major and minor criteria (11). Peutz-Jeghers Syndrome Peutz–Jeghers syndrome is an autosomal dominant condition caused by pathogenic variants in the serine/ threonine kinase 11 (STK11) gene. It is characterized by the presence of two of the following three criteria: 1) two or more hamartomatous polyps throughout the gastro- intestinal tract; 2) mucocutaneous hyperpigmentation of the mouth, lips, nose, eyes

2019 American College of Obstetricians and Gynecologists

235. Discoid lupus erythematosus

pruritic nor painful. Age of onset usually between 20 and 40 years. Lesions often precipitated or aggravated by UV light exposure; smoking also considered a risk factor. Over time, lesions slowly expand, producing areas of peripheral inflammation or hyperpigmentation, leaving a central region of scarring with telangiectasia and hypopigmentation. Diagnosis usually made on clinical grounds, but if in doubt is confirmed with skin biopsy. The mainstay of treatment is topical corticosteroids, or oral (...) . Lupus erythematosus in the 1980s: a survey of 570 patients. Semin Arthritis Rheum. 1991;21:55-64. History and exam disc-shaped erythematous maculopapular scaly lesions age 20 to 40 years hx of UV light exposure smoking hx absence of pruritus and/or pain telangiectasia, hyperpigmentation and/or hypopigmentation permanent scarring alopecia systemic features (arthritis, pleuritis, pericarditis, seizures, psychosis) age 20 to 40 years UV

2017 BMJ Best Practice

236. Congenital adrenal hyperplasia

hydroxysteroid dehydrogenase deficiency. History and exam genetic predisposition weight loss failure to thrive vomiting hypotension ambiguous genitalia hyperpigmentation poor feeding irregular menses infertility male-pattern baldness (females) short stature precocious puberty polycystic ovaries hirsutism severe cystic acne genetic predisposition Diagnostic investigations serum 17-hydroxyprogesterone serum 11-deoxycortisol serum chemistry microfilter paper radioimmunoassay for 17-hydroxyprogesterone genetic

2017 BMJ Best Practice

237. Chronic venous insufficiency

. This most commonly results from venous reflux due to faulty valve function developing as a long-term sequela of DVT and recanalisation and may also develop due to primary valvular incompetence without previous episode(s) of DVT. The term 'CVI' is usually reserved for more advanced disease involving oedema, skin changes, or frank ulcers. History and exam presence of risk factors corona phlebectatica (malleolar flare or ankle flare) ankle swelling hyperpigmentation (brawny oedema) lipodermatosclerosis

2017 BMJ Best Practice

238. Topical medication instillation techniques for glaucoma. Full Text available with Trip Pro

in the fluid removal group compared with the no fluid removal group (MD -1.70 mm, 95% CI -3.46 to 0.06; 10 participants; low-certainty evidence). Fewer eyes showed skin hyperpigmentation in the eyelid region towards the nose in the fluid removal group compared with the no removal group (RR 0.07, 95% CI 0.01 to 0.84; 10 participants; low-certainty evidence); however, the difference was uncertain in the eyelid region towards the temples (RR 0.44, 95% CI 0.07 to 2.66; 10 participants; low-certainty evidence

2017 Cochrane

239. Acne - Guidelines for Prescribing Topical Treatment

Occurs near hair-line cosmetic X XX XX X Occurs where cosmetics used occupational XX X X Excoriated X X Crusts, scar, erosions, hyperpigmentation Mechanical XX XX X occurs when sinus tracts (channels) form between acne lesions resulting in the formation of cysts and abscesses. This type of acne is considered severe and often requires systemic treatment. Suspected cases should be referred to their physician. is an acute eruption of large inflammatory nodules, occurring most frequent in males. It also (...) at their lesions. A hallmark sign of this are scars in the absence of cysts or nodules, or hyperpigmentation of an area that can last years. Mechanical acne is the result of physical irritation to an area leading to the acne lesions, such as a sweat band rubbing against the forehead. Drug-induced acne. Medications that can cause/worsen acne: glucocorticoids (oral, inhaled, and topical), androgens, oral contraceptives containing progestins with more potent androgenic actiivity (norethindrone, levonorgestrel

2018 medSask

240. Guidelines for the Management of Genital, Anal and Throat HPV Infection in New Zealand

with nitrous oxide or carbon dioxide cryoguns. Patients can be referred for treatment. Side effects: Pain and necrosis following application of cryotherapy are fairly universal, and blistering may occur. The treatment of large warts or areas at one time can create wound care problems. Adverse effects include irritation, local oedema, necrosis, ulceration and pain, especially when the treated area thaws. Both hypo- and hyperpigmentation can occur, but this is usually temporary. Although the use of injected (...) . Suitable for: Vulval, vaginal, cervical and perianal warts. Not considered first-line treatment because of expense. Can be considered if there are obstructive or large lesions. 61 Contraindications: None. Can be used in pregnancy. Technique: Advanced training and expertise required. Specialist only. Side effects: Local pain. Scarring and hypo- or hyperpigmentation can be minimised by controlling depth and avoiding treatment beyond the dermal papillae. Trichloroacetic acid (TCA) Mechanism of action: TCA

2017 New Zealand Sexual Health Society

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