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Nail Telangiectasia

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81. Dermatologic Manifestations of Renal Disease (Follow-up)

) dermatologic manifestations of uremia, and (3) dermatologic disorders associated with renal transplantation. The image below illustrates several uremia-related cutaneous disorders. Hands of a transfusion-dependent patient on long-term hemodialysis. Several uremia-related cutaneous disorders are visible. The pigmentary alteration results from retained urochromes and hemosiderin deposition. The large bullae are consistent with either porphyria cutanea tarda or the bullous disease of dialysis. All nails show (...) the distal brown-red and proximal white coloring of half-and-half nails. This article does not discuss systemic disorders, because most of them are discussed in other articles in . The purpose of this article is to integrate renal and cutaneous aspects of disease as well as highlight some important, although frequently underappreciated, clinical or laboratory findings that ally renal and skin diseases. Recognition of the details may provide clinicians with greater insight into the management of patients

2014 eMedicine.com

83. Cirrhosis (Follow-up)

, spider angiomata, skin telangiectasias ("paper money skin"), palmar erythema, white nails, disappearance of lunulae, and finger clubbing, especially in the setting of hepatopulmonary syndrome. Diagnosis Hepatorenal syndrome Hepatorenal syndrome is diagnosed when a creatinine clearance rate of less than 40 mL/min is present or when a serum creatinine level of greater than 1.5 mg/dL, a urine volume of less than 500 mL/day, and a urine sodium level of less than 10 mEq/L are present. [ ] Urine osmolality

2014 eMedicine.com

84. Collagen-Vascular Disease Associated With Interstitial Lung Disease (Follow-up)

severe interstitial fibrosis and medial fibrosis and smooth muscle hyperplasia of pulmonary arteriole, compatible with pulmonary hypertension. Heliotrope rash in woman with dermatomyositis. Gottron papules and nail-fold telangiectasia in patient with dermatomyositis. Classic malar rash (butterfly rash) with distribution over cheeks and nasal bridge. Note that fixed erythema (sometimes associated with mild induration, as here) characteristically spares nasolabial folds. High-resolution CT scan (...) in Collagen-Vascular Diseases CVD Skin and Musculoskeletal System Lungs Heart Salivary Glands Eyes RA * Subcutaneous nodules, digital ulcers, nail-fold infarcts Bibasilar Velcro crackles, signs of pulmonary hypertension, pleural effusion Pericarditis, myocarditis N/A N/A SLE * Malar rash, alopecia, livedo reticularis, erythema, telangiectasia, capillary infarcts, polyarthritis Pleural effusion or rub, pneumonitis, cor pulmonale, diaphragmatic weakness Pericarditis, myocarditis, CAD N/A N/A SD Thickening

2014 eMedicine.com

85. Cirrhosis (Diagnosis)

, spider angiomata, skin telangiectasias ("paper money skin"), palmar erythema, white nails, disappearance of lunulae, and finger clubbing, especially in the setting of hepatopulmonary syndrome. Diagnosis Hepatorenal syndrome Hepatorenal syndrome is diagnosed when a creatinine clearance rate of less than 40 mL/min is present or when a serum creatinine level of greater than 1.5 mg/dL, a urine volume of less than 500 mL/day, and a urine sodium level of less than 10 mEq/L are present. [ ] Urine osmolality

2014 eMedicine.com

86. Drug-Induced Pigmentation (Diagnosis)

antimalarials for at least 4 months will develop bluish-gray or purple pigmentation. See image below. Perioral chloroquine pigmentation. Discoloration appears most frequently in the pretibial areas of the lower extremities but can also involve the entire nail bed, nose, cheeks, forehead, ears, and oral mucosa (specifically the hard palate). Initial lesions manifest as discrete, oval macules before eventually coalescing into large patches; histologic examination of the discolored areas reveals increased (...) cause various adverse cutaneous effects, including photosensitivity and diffuse or localized hyperpigmentation of the skin, nails, and mucous membranes. Individual medications within this group induce a variety of distinctive patterns and colors of dyspigmentation. The pathogenesis underlying chemotherapy-related dyspigmentation is not completely known, but some proposed mechanisms for this hyperpigmentation include direct stimulation of melanin production and postinflammatory hyperpigmentation

2014 eMedicine.com

87. Drug Eruptions (Diagnosis)

of accumulation is argyria (blue-gray discoloration of skin and nails) observed with use of silver nitrate nasal sprays. Adverse effects are normal but unwanted effects of a drug. For example, antimetabolite chemotherapeutic agents, such as cyclophosphamide, are associated with hair loss. The direct release of mast cell mediators is a dose-dependent phenomenon that does not involve antibodies. For example, aspirin and other NSAIDs cause a shift in leukotriene production, which triggers the release (...) , cyclophosphamide, cytarabine, hexamethylene bisacetamide (HMBA), hydroxyurea, imatinib, levamisole, 6-mercaptopurine, MTX, mitoxantrone, rituximab, and tamoxifen Cutaneous reactions to targeted chemotherapy are as follows: Epidermal growth factor receptor inhibitors (eg, gefitinib, cetuximab, erlotinib [ ] - Abnormal scalp, face hair, and/or eyelash growth, anaphylactic infusion reaction (cetuximab), papules and annular plaques, paronychia with/without pyogenic granulomas, telangiectasias, and xerosis. [ ] See

2014 eMedicine.com

89. Collagen-Vascular Disease Associated With Interstitial Lung Disease (Diagnosis)

. . Chartrand S, Fischer A. Management of connective tissue disease-associated interstitial lung disease. Rheum Dis Clin North Am . 2015 May. 41(2):279-94. . Media Gallery Pulmonary hypertension is complication of various collagen-vascular diseases. Lung biopsy specimen demonstrates severe interstitial fibrosis and medial fibrosis and smooth muscle hyperplasia of pulmonary arteriole, compatible with pulmonary hypertension. Heliotrope rash in woman with dermatomyositis. Gottron papules and nail-fold (...) telangiectasia in patient with dermatomyositis. Classic malar rash (butterfly rash) with distribution over cheeks and nasal bridge. Note that fixed erythema (sometimes associated with mild induration, as here) characteristically spares nasolabial folds. High-resolution CT scan of advanced-stage pulmonary fibrosis demonstrating reticular opacities with honeycombing in predominantly subpleural distribution. This pattern can be present in rheumatoid arthritis–related interstitial lung disease, Sjögren syndrome

2014 eMedicine.com

90. Dermatologic Manifestations of Pulmonary Disease (Diagnosis)

decades (see the image below). Lesions typically range from one to several millimeters in diameter, but they may grow much larger. Three-dimensional reconstructions of serial sections of skin biopsy specimens have established these lesions to be arteriovenous malformations. Hereditary hemorrhagic telangiectasia. Photographs show characteristic telangiectasias of the lips and tongue (left) and nail (right). Treatment The treatment of PAVMs consists of surgical resection or obliteration of the shunt (...) by interstitial edema and infiltration by plasma cells and lymphocytes. Connective-tissue proliferation and interstitial edema result in a characteristic spongy quality to the proximal nail fold—as if the nail were floating in the nail bed. Clinical findings Clinical examination findings may correlate with the severity of clubbing. The earliest finding may be the Schamroth sign, first described by a South African physician when he developed clubbing secondary to infective endocarditis. [ ] In healthy

2014 eMedicine.com

91. Dermatologic Manifestations of Hematologic Disease (Diagnosis)

Manifestations of Anemia Pallor of the skin and mucocutaneous membranes can be a sign of severe anemia of any cause. Severe anemia (hemoglobin Iron deficiency anemia can cause changes in the appearance of the nails, tongue, and hair. In iron deficiency, nails become fragile and develop longitudinal ridges. Nail-plate alterations follow, with the development of koilonychia, a spoonlike convexity (see the images below). Koilonychia. Koilonychia. In iron deficiency anemia, reduced oxygen availability (...) is believed to diminish disulfide bond formation, which consequently reduces nail-plate pliability. Changes also occur in the oral mucosa and on the tongue. Of 378 patients with iron deficiency, 14% had angular stomatitis and almost half had alterations in tongue papillae, either a change to the filiform type or atrophy. [ , ] Diffuse hair thinning is reported in female blood donors and is believed to be caused by a decrease in the iron storage pool. Changes in hair quality, primarily increased splitting

2014 eMedicine.com

92. Dermatologic Manifestations of Cardiac Disease (Diagnosis)

Clubbing (Hypertrophic Osteoarthropathy) Definition represents a localized drumsticklike swelling of the distal segments of fingers and toes, particularly over the extensor surface. It is caused by connective tissue proliferation leading to increases in the sponginess of the soft tissue at the base of the nails due to stimulation by a humoral substance that causes dilation of the vessels of the fingertip or toe tip. [ ] Differential diagnosis Clubbing, as illustrated below, is seen in persons (...) of clubbing of a finger in a patient with Eisenmenger syndrome (right-to-left shunt). Cyanosis Definition is a bluish discoloration of the skin and mucous membranes due to an increased amount of reduced hemoglobin in the small blood vessels of the skin. It is most appreciable in the lips, nail beds, earlobes, and cheeks. The mechanism includes either dilatation of cutaneous venules or a reduction in the oxygen saturation of intracapillary blood. Cyanosis manifests when the absolute concentration

2014 eMedicine.com

93. Dermoscopy (Diagnosis)

% for the examination by naked eye and 90% for dermoscopy. The equipment; technologic methods; diagnostic features; and primary aspects of melanoma, common nevi, atypical nevi, and other nonmelanocytic pigmented skin lesions are discussed in this article. The technique is also commonly used to assist in the evaluation of nail fold capillaries and features of alopecia. [ , ] Next: Technical Procedures and Equipment Dermoscopy involves an evaluation of the skin surface. During a dermoscopy assessment, the pigmented (...) occur in white areas that correspond to well-defined white or milky red-white areas. In contrast to hypopigmented areas, depigmented areas completely lack pigment. Histologically, they correspond to fibroplasia, telangiectasias, and loss of melanin. See images below. Hypopigmented areas in a melanocytic nevus. White areas in invasive melanoma. Negative pigment network Negative pigment network is seen as a negative of the pigmented network with relatively lighter serpiginous lines making up

2014 eMedicine.com

94. Dermatologic Manifestations of Gastrointestinal Disease (Diagnosis)

macular or papular, sharply demarcated telangiectases on the face, lips, palate, tongue, ears, chest, or extremities, with occasional presentation under nails. The age of onset for the telangiectases is most often the third decade of life, although earlier presentations may occur during adolescence. Although the distribution of lesions and associated bleeding diathesis are clinically suggestive of hereditary hemorrhagic telangiectasia, it may occasionally be difficult to distinguish from similar (...) within the GI tract. This review attempts to explore this relationship by describing disorders involving both the GI tract and the skin. [ , ] Table 1. Dermatologic Manifestations of Alimentary Disorders Dermatologic Manifestation GI Abnormality Disorder Periorificial granulomas [ ] Malabsorption Crohn disease Koilonychia Esophageal webs Plummer-Vinson syndrome Liver disease Hemochromatosis Palmoplantar keratoderma Esophageal carcinoma Howel-Evans syndrome Acral rash Bazex syndrome [ ] Telangiectasia

2014 eMedicine.com

95. Dermatologic Manifestations of Renal Disease (Diagnosis)

) dermatologic manifestations of uremia, and (3) dermatologic disorders associated with renal transplantation. The image below illustrates several uremia-related cutaneous disorders. Hands of a transfusion-dependent patient on long-term hemodialysis. Several uremia-related cutaneous disorders are visible. The pigmentary alteration results from retained urochromes and hemosiderin deposition. The large bullae are consistent with either porphyria cutanea tarda or the bullous disease of dialysis. All nails show (...) the distal brown-red and proximal white coloring of half-and-half nails. This article does not discuss systemic disorders, because most of them are discussed in other articles in . The purpose of this article is to integrate renal and cutaneous aspects of disease as well as highlight some important, although frequently underappreciated, clinical or laboratory findings that ally renal and skin diseases. Recognition of the details may provide clinicians with greater insight into the management of patients

2014 eMedicine.com

96. Epistaxis (Follow-up)

Hemorrhagic Telangiectasia Management of hereditary hemorrhagic telangiectasia (HHT) is palliative because the underlying defect is not curable. Options include coagulation with potassium-titanyl-phosphate (KTP) or neodymium:yttrium-aluminum-garnet (Nd:YAG) lasers, septodermoplasty, embolization, and estrogen therapy. [ ] A study by Wirsching et al indicated that in patients with HHT, supplementing Nd:YAG laser therapy with temporary nasal occlusion with hypoallergenic tape can reduce the Epistaxis (...) activities - Protection from direct trauma from some sports activities is afforded by the use of helmets or face pieces. Hot and dry environments – The effects of such environments can be mitigated by using humidifiers, better thermostatic control, saline spray, and antibiotic ointment on the Kiesselbach area. Hot and spicy foods Digital trauma – In children, nose picking is difficult to deter and should probably be considered inevitable. Keeping the child’s nails well trimmed may be helpful. Nose

2014 eMedicine Pediatrics

97. Colorectal Tumors (Follow-up)

pigmentation of lips, oral mucosa, and perioral region Cronkhite-Canada syndrome - GI polyposis, skin hyperpigmentation, alopecia, and nail changes Osler-Weber-Rendu syndrome - Juvenile polyps and hepatic telangiectasia Oldfield syndrome - Polyposis and multiple sebaceous cysts Bloom syndrome - Growth retardation, accelerated aging, immune deficiency, and malignant tumors Cowden syndrome - Hamartomas, GI polyps, breast, thyroid, and GI cancer Ruvalcaba-Myhre-Smith syndrome - Microcephaly and juvenile (...) in which the GI polyps are associated with skin hyperpigmentation, alopecia, and nail changes (Cronkhite, 1955). Hair loss, and skin and nail changes may be evident long before GI symptoms appear. The hamartomatous polyps appear in the stomach and colon. Chronic diarrhea results in malabsorption, hypovitaminosis, hypoproteinemia, and fluid and electrolyte imbalance. Because patients with Cronkhite-Canada syndrome may develop colonic malignancy, close follow-up is recommended (see Syndromes associated

2014 eMedicine Pediatrics

98. Colorectal Tumors (Overview)

pigmentation of lips, oral mucosa, and perioral region Cronkhite-Canada syndrome - GI polyposis, skin hyperpigmentation, alopecia, and nail changes Osler-Weber-Rendu syndrome - Juvenile polyps and hepatic telangiectasia Oldfield syndrome - Polyposis and multiple sebaceous cysts Bloom syndrome - Growth retardation, accelerated aging, immune deficiency, and malignant tumors Cowden syndrome - Hamartomas, GI polyps, breast, thyroid, and GI cancer Ruvalcaba-Myhre-Smith syndrome - Microcephaly and juvenile (...) in which the GI polyps are associated with skin hyperpigmentation, alopecia, and nail changes (Cronkhite, 1955). Hair loss, and skin and nail changes may be evident long before GI symptoms appear. The hamartomatous polyps appear in the stomach and colon. Chronic diarrhea results in malabsorption, hypovitaminosis, hypoproteinemia, and fluid and electrolyte imbalance. Because patients with Cronkhite-Canada syndrome may develop colonic malignancy, close follow-up is recommended (see Syndromes associated

2014 eMedicine Pediatrics

99. Colorectal Tumors (Treatment)

pigmentation of lips, oral mucosa, and perioral region Cronkhite-Canada syndrome - GI polyposis, skin hyperpigmentation, alopecia, and nail changes Osler-Weber-Rendu syndrome - Juvenile polyps and hepatic telangiectasia Oldfield syndrome - Polyposis and multiple sebaceous cysts Bloom syndrome - Growth retardation, accelerated aging, immune deficiency, and malignant tumors Cowden syndrome - Hamartomas, GI polyps, breast, thyroid, and GI cancer Ruvalcaba-Myhre-Smith syndrome - Microcephaly and juvenile (...) in which the GI polyps are associated with skin hyperpigmentation, alopecia, and nail changes (Cronkhite, 1955). Hair loss, and skin and nail changes may be evident long before GI symptoms appear. The hamartomatous polyps appear in the stomach and colon. Chronic diarrhea results in malabsorption, hypovitaminosis, hypoproteinemia, and fluid and electrolyte imbalance. Because patients with Cronkhite-Canada syndrome may develop colonic malignancy, close follow-up is recommended (see Syndromes associated

2014 eMedicine Pediatrics

100. Epistaxis (Treatment)

Hemorrhagic Telangiectasia Management of hereditary hemorrhagic telangiectasia (HHT) is palliative because the underlying defect is not curable. Options include coagulation with potassium-titanyl-phosphate (KTP) or neodymium:yttrium-aluminum-garnet (Nd:YAG) lasers, septodermoplasty, embolization, and estrogen therapy. [ ] A study by Wirsching et al indicated that in patients with HHT, supplementing Nd:YAG laser therapy with temporary nasal occlusion with hypoallergenic tape can reduce the Epistaxis (...) activities - Protection from direct trauma from some sports activities is afforded by the use of helmets or face pieces. Hot and dry environments – The effects of such environments can be mitigated by using humidifiers, better thermostatic control, saline spray, and antibiotic ointment on the Kiesselbach area. Hot and spicy foods Digital trauma – In children, nose picking is difficult to deter and should probably be considered inevitable. Keeping the child’s nails well trimmed may be helpful. Nose

2014 eMedicine Pediatrics

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