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Chapter 054. Skin Manifestations of Internal Disease (Part 1)

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Skin Manifestations of Internal Disease. Skin Manifestations of Internal Disease: Introduction. It is now a generally accepted concept in medicine that the skin can show signs of internal disease. Therefore, in textbooks of medicine one finds a chapter describing in detail the major systemic disorders that can be identified by cutaneous signs.

Chapter 054. Skin Manifestations of Internal Disease (Part 27)

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Braverman IM: Skin Signs of Systemic Disease, 3d ed. Philadelphia, Saunders, 1998. Callen JP et al: Dermatological Signs of Internal Disease, 3d ed.. Philadelphia, Saunders, 2003. McKee PH et al: Pathology of the Skin, 3d ed. Spitz JL: Genodermatoses: A Clinical Guide to Genetic Skin Disorders, 2d ed

Chapter 054. Skin Manifestations of Internal Disease (Part 19)

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The presence of multiple tricholemmomas on the face and cobblestoning of the oral mucosa points to the diagnosis of Cowden disease (multiple hamartoma syndrome) due to mutations in the PTEN gene.. Internal organ involvement (in decreasing order of frequency) includes fibrocystic disease and carcinoma of the breast, adenomas and carcinomas of the thyroid, and gastrointestinal polyposis. Keratoses of the palms, soles, and dorsal aspect of the hands are also seen.

Chapter 054. Skin Manifestations of Internal Disease (Part 22)

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In the majority of these patients, however, the internal involvement remains indolent. The papules coalesce into plaques on the extensor surfaces of knees, elbows, and the small joints of the hand.

Chapter 054. Skin Manifestations of Internal Disease (Part 10)

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Seborrheic keratoses are common lesions, but in one rare clinical setting they are a. sign of systemic disease, and that setting is the sudden appearance of multiple lesions, often with an inflammatory base and in association with acrochordons (skin tags) and acanthosis nigricans. This is termed the sign of Leser-Trélat and alerts the clinician to search for an internal malignancy.

Chapter 054. Skin Manifestations of Internal Disease (Part 9)

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Patients with vitiligo also have an increased incidence of several autoimmune disorders, including hypothyroidism, Graves' disease, pernicious anemia, Addison's disease, uveitis, alopecia areata, chronic mucocutaneous candidiasis, and the polyglandular autoimmune syndromes (types I and II). Diseases of the thyroid gland are the most frequently associated disorders, occurring in up to 30% of patients with vitiligo.

Chapter 054. Skin Manifestations of Internal Disease (Part 17)

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Solar urticaria characteristically occurs within minutes of sun exposure and is a skin sign of one systemic disease—. In addition to the urticaria, these patients have subtle pitted scarring of the nose and hands. Cold urticaria is precipitated by exposure to the cold, and therefore exposed areas are usually affected. In some patients, the disease is associated with abnormal circulating proteins—more commonly cryoglobulins and less commonly cryofibrinogens or cold agglutinins..

Chapter 054. Skin Manifestations of Internal Disease (Part 8)

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Poikiloderma does not imply a single disease entity—although becoming less common, it is seen in skin damaged by ionizing radiation as well as in patients with autoimmune connective tissue diseases, primarily dermatomyositis (DM), and rare genodermatoses (e.g., Kindler syndrome).. In scleroderma, the dilated blood vessels have a unique configuration and are known as mat telangiectasias.

Chapter 054. Skin Manifestations of Internal Disease (Part 3)

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There have been isolated case reports of erythroderma secondary to some solid tumors—lung, liver, prostate, thyroid, and colon—but it is usually in a late stage of the disease.. (Table 54-4) The two major forms of alopecia are scarring and nonscarring.. In scarring alopecia there are associated fibrosis, inflammation, and loss of hair follicles.

Chapter 054. Skin Manifestations of Internal Disease (Part 12)

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Approximately 50% of the patients with Whipple's disease have an associated generalized hyperpigmentation in association with diarrhea, weight loss, arthritis, and lymphadenopathy. Of the autoimmune diseases associated with diffuse hyperpigmentation, biliary cirrhosis and scleroderma are the most common, and occasionally both disorders are seen in the same patient. The skin is dark brown in color, especially in sun-exposed areas.

Chapter 054. Skin Manifestations of Internal Disease (Part 5)

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Scarring alopecia is more frequently the result of a primary cutaneous disorder such as lichen planus, folliculitis decalvans, chroniccutaneous (discoid) lupus, or linear scleroderma (morphea) than it is a sign of systemic disease.. Although the scarring lesions of discoid lupus can be seen in patients with systemic lupus, in the majority of cases the disease process is limited to the skin.. Less common causes of scarring alopecia include sarcoidosis (see "Papulonodular Skin Lesions,".

Chapter 054. Skin Manifestations of Internal Disease (Part 25)

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Peripheral vascular disease (Chap. Infections, e.g., ecthyma caused by Streptococcus (Chap. Cutaneous small-vessel vasculitis b. Antiphospholipid syndrome (Chap. Raynaud's phenomenon (Chap. Infections, e.g., dimorphic fungi, chronic herpes simplex. Behçet's syndrome (Chap

Chapter 054. Skin Manifestations of Internal Disease (Part 13)

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Bullous dermatosis of hemodialysis b. c Associated with gluten enteropathy.. d Associated with inflammatory bowel disease.

Chapter 054. Skin Manifestations of Internal Disease (Part 16)

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A similar desquamation of the palms and soles is seen in toxic shock syndrome (TSS), Kawasaki's disease, and after severe febrile illnesses. 129), and three of these involve mucocutaneous sites (diffuse erythema of the skin, desquamation of the palms and soles 1–2 weeks after onset of illness, and involvement of the mucous membranes).

Chapter 054. Skin Manifestations of Internal Disease (Part 6)

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Adrenal origin, e.g., Cushing's disease, 21-hydroxylase deficiency. Ovarian origin, e.g., polycystic ovary syndrome. Patients with the carcinoid syndrome have episodes of flushing of the head, neck, and sometimes the trunk. Resultant skin changes of the face, in particular telangiectasias, may mimic the clinical appearance of acne rosacea.. Acneiform eruptions (see "Acne,". above) and folliculitis represent the most common pustular dermatoses.

Chapter 054. Skin Manifestations of Internal Disease (Part 20)

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The internal manifestations of Torre syndrome include multiple carcinomas of the gastrointestinal tract (primarily colon) as well as cancers of the larynx, genitourinary tract, and endometrium.

Chapter 054. Skin Manifestations of Internal Disease (Part 24)

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Purpura secondary to cholesterol emboli are usually seen on the lower extremities of patients with atherosclerotic vascular disease. They often follow anticoagulant therapy or an invasive vascular procedure such as an arteriogram but also occur spontaneously from disintegration of atheromatous plaques.. Multiple step sections of the biopsy specimen may be necessary to demonstrate the cholesterol clefts within the vessels.

Chapter 054. Skin Manifestations of Internal Disease (Part 23)

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Blue Lesions. Lesions that are blue in color are the result of either vascular ectasias and tumors or melanin pigment in the dermis. Venous lakes (ectasias) are compressible dark-blue lesions that are found commonly in the head and neck region. Venous malformations are also compressible blue papulonodules and plaques that can occur anywhere on the body, including the oral mucosa....

Chapter 054. Skin Manifestations of Internal Disease (Part 21)

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Widespread acute eruptions of erythematous papules are discussed in the section on exanthems.. culture of lesional tissue will aid in the diagnosis.. The diseases that are characterized by erythematous plaques with scale are reviewed in the papulosquamous section, and the various forms of dermatitis are discussed in the section on erythroderma. Additional disorders in the differential diagnosis of red papules/plaques...

Chapter 054. Skin Manifestations of Internal Disease (Part 4)

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The most common causes of nonscarring alopecia include telogen effluvium, androgenetic alopecia, alopecia areata, tinea capitis, and some cases of traumatic alopecia (Table 54-5). In women with androgenetic alopecia, an elevation in circulating levels of androgens may be seen as a result of ovarian or adrenal gland dysfunction. When there are signs of virilization, such as a deepened voice and...