Sec 26 Skin Manifestations of Internal Organ Disorders

Question 1

Hyperelastic velvety skin that rebounds to the original position after being stretched, “cigarette-paper” scars, and hyperextensible joints; mitral and tricuspid prolapse, dilatation of the aorta and pulmonary artery, arterial rupture, myocardial infarction, and emphysema may accompany this syndrome

Answer 1

Ehlers-Danlos syndrome

Question 2

Progressive looseness of the skin with pendulous folds and droopy eyelids may be associated with generalized hyperelastosis leading to aortic dilatation and rupture, congestive heart failure, or cor pulmonale with pulmonary artery stenosis and progressive emphysema

Answer 2

Cutis laxa

Question 3

Skin is thick, lax, and yellowish, especially over the axillae, antecubital area, and neck; yellow patches may occur on mucous membranes, especially the labia; alterations in the ABCC6 gene may also lead to arteries becoming calcified, the aortic and mitral valves thickened, and cardiovascular symptoms, such as angina pectoris and claudication are frequent symptoms

Answer 3

Pseudoxanthoma elasticum (PXE)

Question 4

Skin appears atrophic and tight from a very early age with marked loss of subcutaneous tissue and leg ulceration; coronary atherosclerosis frequently leads to premature death by myocardial infarction

Answer 4

Hutchinson-Gilford syndrome (progeria)

Question 5

The skin is pale due to anemia and often exhibits a distinctive muddy hue, due to accumulation of carotenoid and nitrogenous pigments (urochromes) in the dermis.

Answer 5

Chronic Renal Failure

Question 6

An increase in the absolute amount of desaturated (reduced) hemoglobin results in a purple–blue discoloration of the skin.

Answer 6

Cyanosis

Question 7

Develops when there is normal arterial oxygen saturation but reduced blood flow, such as low-output cardiac failure and local vasoconstriction. Seen on cooler areas such as the nose, lips, earlobes, and fingertips.

Answer 7

Peripheral cyanosis

Question 8

Occurs in states that produce low arterial oxygen saturation, such as congenital heart disease with intracardiac or intrapulmonary right-to-left shunting, or severe lung disease. Usually visible on warm areas of the skin like the tongue, oral mucosae, and conjunctivae.

Answer 8

Central cyanosis

Question 9

Caused by an increase in the amount of saturated hemoglobin, an increase in the diameter or actual number of skin capillaries, or a combination of these factors.

Answer 9

Erythema

Question 10

This is most pronounced on the tongue, lips, nose, earlobes, conjunctivae, and fingertips. It is due to increased amounts of saturated hemoglobin producing erythema with increased amounts of desaturated hemoglobin producing cyanosis because of the inability of the body to fully oxygenate the increased absolute amounts of hemoglobin.

Answer 10

Polycythemia

Question 11

Paroxysmal intense condition of the face, neck, chest, and abdomen, often with telangiectases of the face and neck, may occur in patients with carcinoid tumors, systemic mastocytosis, and pheochromocytoma, alone or in Sipple syndrome.

Answer 11

Flushing

Question 12

Common in primary biliary cirrhosis (PBC) and may be an early presenting sign. It initially involves exposed areas, but gradually becomes generalized. Blotchy, circumscribed areas of dirty brown pigmentation are also occasionally evident.

Answer 12

Melanosis

Question 13

A condition in which small white macules, sometimes with a central spider, appear on the skin of the buttocks, back, thighs, and forearms, may occur in cirrhosis and, rarely, in PBC.

Answer 13

Guttate hypomelanosis

Question 14

The generalized metallic gray or bronze-brown color of the skin. There is accentuation in sunexposed and traumatized skin, and occasionally there is buccal and conjunctival pigmentation.

Answer 14

Hemochromatosis

Question 15

Gives rises to lichenified, and often deeply pigmented skin in sunexposed sites and can develop in patients with alcoholic liver disease.

Answer 15

Pellagra

Question 16

Causes pigmentation of the distal extremities in a glove and stocking distribution.

Answer 16

Vitamin B 12 deficiency

Question 17

Results from increased cellular or connective tissue binding of bilirubin and its metabolites in the skin producing a generalized coloration of the skin, mucous membranes, and other body tissues varying in hue from faint golden to dark green-yellow. Results from an imbalance between tissue production and hepatic clearance of bilirubin.

Answer 17

Jaundice

Question 18

Yellow skin pigmentation due to ingestion of tomato juice.

Answer 18

Lycopenemia

Question 19

One of the most common and distressing symptoms of hepatobiliary disease. More commonly occurs in conditions causing cholestasis and range from mild and transient to severe and prolonged. Usually most marked on the extremities and only rarely involves the neck and face, and genitalia.

Answer 19

Pruritus

Question 20

Neurotransmitter receptor antagonist which has been advocated for the pruritus of cholestasis

Answer 20

Nalmefene

Question 21

Has been thought to be caused by a combination of increased serum histamine, vitamin A, and parathyroid hormone (PTH); mast cell hyperplasia; peripheral polyneuropathy; xerosis; and inflammatory factors. Clinically, the skin may appear normal or demonstrate a variety of lichenified or hyperkeratotic lesions.

Answer 21

Renal pruritus

Question 22

Excessive growth of lanugo hair is a rare complication of gastrointestinal cancer.

Answer 22

Hypertrichosisalas Lanuginosa

Question 23

One of the amine precursor uptake and decarboxylation (APUD) cell tumors, usually arises in the islet cells of the pancreas, and occurs in association with a distinctive necrolytic migratory erythema.

Answer 23

Glucagonoma

Question 24

Are normal in their distal 50% and white in the proximal 50% and found in CRF.

Answer 24

Lindsey, or Half-and-half, nails

Question 25

Flushing of the nail beds that is synchronous with the heartbeat which is a sign of aortic regurgitation.

Answer 25

Quincke pulsation

Question 26

Intensely white nails characteristic of cirrhosis. Thumbs and forefingers are often most affected. The whiteness does not alter with nail growth or with compression of digital vessels and is thought to be due to the opacity of the nail plate itself. The so-called watch-glass deformity may accompany white nails.

Answer 26

Terry nails

Question 27

A bluish color of the lunular portion of nails, occurs in hepatolenticular degeneration (Wilson disease)

Answer 27

Azure lunulae

Question 28

MC in: Nail clubbing

Answer 28

Bronchogenic carcinoma
Suppurative lung disease
Endocarditis
Congenital heart disease

Question 29

Beaking or distal curvature of the nail. There may be loss of the angle between the nail and cuticle. Sponginess or “floating” of the nail when pressure is applied is also characteristic and the size of the terminal tuft may increase.
Anatomically, there is an increased thickness of the nail bed.

Answer 29

Nail clubbing

Question 30

Yellow nail syndrome

Answer 30

Yellow nails
Primary lymphedema
Pleural effusion(s)

Question 31

The characteristics of the nails include thickening, transverse ridging, diminished growth, increased curvature with a “hump,” and onycholysis. The lunulae and cuticles may be absent. The color may vary from pale yellow to green. The nail changes are secondary to congenitally hypoplastic lymphatics, leading to lymphedema, which is characteristically slowly progressive and somewhat asymmetric, with induration and hyperkeratosis extending to the thighs. Periodic lymphangitis is frequent and may contribute to the swelling.

Answer 31

Yellow nail syndrome

Question 32

A chronic clinical syndrome that is characterized by recurrent attacks of burning pain in the hands and/or feet with redness and warmth. There may be swelling that extends to the elbows and knees. May be primary or secondary to a disorder such as atherosclerosis, hypertension, and polycythemia. Symptoms are thought to be caused by vascular dysfunction including arteriovenous shunting and reduced capillary perfusion with subsequent tissue hypoxemia.

Answer 32

Erythromelalgia (labile hyperthermia)

Question 33

A primary form of HOA, is frequently familial with an onset around puberty, and has a number of cutaneous manifestations. Distinctive thickening and furrowing of the skin of the scalp, forehead, and cheeks may create leonine facies.

Answer 33

Pachydermoperiostosis

Question 34

There is an exaggeration of normal mottling; the hands are warm and bright red, especially on the palm, the dorsae of the hands, the fingers, and the nail bases. Or there is well-demarcated redness of the hypothenar eminence that gradually spreads to the fingertips and then to other areas of the palm.

Answer 34

Palmar erythema

Question 35

The most representative and classic vascular lesion of chronic liver disease. Clinically, it has a central arteriole from which numerous small, twisted vessels radiate. They range in size from a pinhead to 2 cm. Larger lesions can be seen to pulsate when pressed with a glass slide. Pressure over the central arteriole causes blanching of the whole lesion. These are characterized by the abnormal permanent dilatation of end vessels. Histologically, they consist of a central ascending arteriole ending in a thin-walled ampulla.

Answer 35

Spider nevus

Question 36

Tortuous small arteries that traverse the margins of the ocular sclerae that develop in patients with chronic liver disease.

Answer 36

Corkscrew scleral vessels

Question 37

A postcricoid web is associated with koilonychia, angular stomatitis, a sore tongue, and, usually, iron deficiency in this syndrome where up to 10% of patients with a postcricoid web develop carcinoma at the site.

Answer 37

Plummer–Vinson (Paterson–Kelly) syndrome

Question 38

Characterized by inflammation and destruction of cartilage and connective tissues, including those of the cardiopulmonary system. It is idiopathic but frequently associated with other immunologic disorders such as systemic lupus erytematosus (SLE). Auricular chondritis with pain, swelling, and redness of the pinna but complete sparing of the lobes is characteristic.

Answer 38

Relapsing polychondritis

Question 39

A form of proteinenergy malnutrition, causes hair to become hypopigmented, varying from red–yellow to white, and curly. There may be alternating bands of pale and dark hair.

Answer 39

Kwashiorkor

Question 40

This is an autosomal recessive mutation in desmoplakin, a desmosomal protein important in cell adhesion. Generalized striate keratodermas are seen, particularly on the palmoplantar epidermis, with woolly hair and dilated left ventricular cardiomyopathy leading to arrhythmias, heart failure, and early sudden death

Answer 40

Naxos Disease

Question 41

The most common primary tumors of the heart. The cardiopulmonary findings include, in order of frequency, congestive heart failure, mitral murmur, chest pain, pulmonary edema, and pulmonary emboli. The cutaneous manifestations include biphasic digital color changes on cold exposure, skin lesions may simulate collagen vascular disease or vasculitis with tender, violaceous, nonblanching, annular, and serpiginous lesions of the digital pads, as well as splinter hemorrhages presenting as a systemic vasculitis or infective endocarditis. Characteristic pruritic, erythematous papules as well as cyanosis and ecchymosis of the extremities may also occur.

Answer 41

Atrial myxoma

Question 42

An autosomal dominant disorder comprising lentigines; electrocardiogram conduction defects; ocular hypertelorism; pulmonary stenosis; abnormalities of genitalia; retardation of growth and sensorineural deafness. Electrocardiographic features include axis deviation; prolonged P–R interval, bundle branch block, and complete heart block.

Answer 42

LEOPARD Syndrome

Question 43

Exquisitely painful, tender, and located distally on the digital tufts seen in Bacterial endocarditis. Histologically, are a perivasculitis or necrotizing vasculitis without microabscess formation or other evidence of infection or emboli.

Answer 43

Osler nodes

Question 44

Nontender lesions and located proximally on the palms and soles seen in Bacterial endocarditis. Histologically, they are vasculitis with microabscess formation.

Answer 44

Janeway lesions

Question 45

Acute or chronic pericarditis is an important feature of this. Pericardial effusions may be occult, and constrictive pericarditis and tamponade may occur. Additionally, small-vessel involvement of the myocardium may lead to fibrosis which may cause arrhythmias and sudden death.

Answer 45

Systemic sclerosis

Question 46

An autosomal dominant trait, characterized clinically by punctate, linear, or spider-like telangiectasias of the skin, especially on the upper body, oral and nasal mucous membranes, and nail beds. Radiating arms about an elevated punctum are the most characteristic feature, especially on the lips and tongue. They are distinguished from spider nevi in that they do not pulsate. Recurrent epistaxis, often beginning in childhood or adolescence, is the most frequent presenting symptom.

Answer 46

Osler–Weber–Rendu disease

Question 47

May cause mild respiratory symptoms and be associated with transient pulmonary infiltrates and a peripheral eosinophilia called Löffler syndrome. Skin lesions may present as patchy urticaria or erythematous papular eruptions.

Answer 47

Cutaneous larva migrans,

Question 48

Petechiae, respiratory insufficiency, and cerebral dysfunction after long bone fracture. The petechiae are most common on the neck, axillae, shoulders, chest, and conjunctivae. They often appear before other manifestations on the second or third day after injury, and appear in crops. When widespread, they herald more significant cerebral and pulmonary dysfunction. Histologically, fat globules are present within dermal and pulmonary vessels.

Answer 48

Fat embolism syndrome

Question 49

Skin changes include confluent, waxy papules, plaques, and scarring, particularly on the face. The tongue is enlarged. Oropharyngeal and laryngeal mucous membranes are usually affected early in the course of the disease, during infancy or childhood. Laryngeal infiltration may present early on as hoarsness, and may progress to obstruction and respiratory insufficiency. The trachea and main stem bronchus may be thickened and studded with warty projections.

Answer 49

Lupoid Proteinosis (Hyalinosis Cutis et Mucosae)

Question 50

An uncommon disorder presenting with characteristic mucocutaneous lesions consisting of deeply set, firm, small papules in association with a deforming arthritis, fever, malaise, weight loss, and myopathy. Neurofibroma-like, soft, sessile, larger lesions have been reported, as has an association with malignancy. Cardiopulmonary complications include pleural effusions, pulmonary infarctions, pericarditis, angina pectoris, myocardial infarction, and congestive heart failure.

Answer 50

Multicentric reticulohistiocytosis

Question 51

May cause waxy induration of the skin, with hemorrhagic skin changes on stroking (pinch purpura). Amyloid deposition within the vessels is found in clinically normal skin in up to 50% of patients. Cutaneous involvement occurs most often in the primary and myeloma-associated types, and therefore serves as a marker for cardiopulmonary involvement. Congestive heart failure with cardiomegaly or a restrictive cardiomyopathy occurs. Pathologically, there is infiltration of the endocardium, myocardium, pericardium, valves, and coronary vessels.

Answer 51

Systemic amyloidosis

Question 52

A rare angiocentric and angiodestructive, Epstein–Barr virus-associated B cell lymphoproliferative disorder, varying from an indolent process to an aggressive large cell lymphoma. The skin is the extrapulmonary organ most commonly involved. The disease also affects the lungs, heart, central nervous system, and kidneys. Although many patients remain asymptomatic, cough, dyspnea, and chest pain may occur. Chest X-rays show transient infiltrates which may progress to nodules that cavitate and cause profuse hemoptysis.

Answer 52

Lymphomatoid granulomatosis

Question 53

Lung involvement in SLE

Answer 53

1. Diffuse interstitial pneumonitis
2. Acute pneumonitis
3. Intrapulmonary hemorrhage
4. Diaphragm dysfunction with decreased lung volume
5. Pulmonary hypertension with cor pulmonale
6. Fibrosing alveolitis

Question 54

In patients with cirrhosis portal-systemic collateral vessels may be seen and are a clue to the existence of portal hypertension. Often, the umbilical vein is dilated and visible in the epigastrium.

Answer 54

Caput medusae

Question 55

The cutaneous marker for several inherited lysosomal disorders, due to deficiency of a number of enzymes, notably α-galactosidase A (the cause in Anderson–Fabry disease). Signs include severe, painful neuropathy; progressive renal, cardiovascular, and cerebrovascular dysfunction. Gastrointestinal symptoms include abdominal pain and diarrhea. The diagnosis may be missed without the skin signs.

Answer 55

Angiokeratoma corporis diffusum

Question 56

Palpable purpura, especially on the legs and buttocks, and may occur in conjunction with joint swelling. Up to 76% of patients have gastrointestinal involvement, ranging from colicky abdominal pain, nausea, and vomiting to bloody diarrhea, intussusception, and pancreatitis. Renal involvement is common, especially in patients with bloody stools. Upper gastrointestinal endoscopy reveals multiple raised erythematous lesions or ulceration that resolves after systemic treatment with glucocorticoids.

Answer 56

Henoch–Schönlein purpura

Question 57

An idiopathic, systemic, obliterative vasculopathy. The characteristic skin lesions are recurrent crops of porcelain-white atrophic papules with an elevated erythematous border. These may have been present for years with no other apparent disease although gut vessels are involved in half of the cases. Clinically bleeding, abdominal pain, diarrhea, malabsorption, and bowel perforation with peritonitis occur.

Answer 57

Malignant atrophic papulosis

Question 58

A nonneoplastic tumor composed of tissue elements normally present in that organ.

Answer 58

Hamartoma

Question 59

An autosomal dominant disorder characterized by cutaneous cysts and premalignant adenomatous polyps, particularly in the colorectum. Duodenal and hepatic carcinoma can also occur. The cutaneous lesions frequently predate the gastrointestinal lesions and include solitary or multiple epidermoid cysts, fibromas, lipomas, pilomatricomas, facial osteomas, and distinctive ocular lesions.

Answer 59

Gardner syndrome

Question 60

Autosomal dominant and characterized by small darkly pigmented macules around the mouth, on the lips, buccal mucosa, and digits. A histologically unique type of hamartomatous polyp occurs, mainly in the small intestine, which predisposes to recurrent intussusception. Bleeding is relatively rare, but up to 2% of patients develop adenocarcinoma of the stomach, duodenum, and colon. There is also an increased risk of malignancy in general.

Answer 60

Peutz–Jeghers syndrome

Question 61

There is patchy alopecia and characteristic, nail changes. Inflammatory polyps are present in the stomach and bowel, and abdominal pains with a severe protein-losing enteropathy are common.

Answer 61

Cronkhite-Canada syndrome

Question 62

Oral granulomatous nodules are common where they may coalesce to give a “cobblestone” appearance. It is one cause of granulomatous cheilitis, which may predate bowel disease by several years. Granulomas may also occur in the perineum, at colostomy and ileostomy sites, and in association with scars, sinuses, and fistulas. Very rarely, granulomas occur at sites not contiguous with the bowel, such as the trunk and limbs.

Answer 62

Crohn disease

Question 63

Bruising of the skin around the umbilicus in acute pancreatitis

Answer 63

Cullen sign

Question 64

Bruising of the skin n the flanks in acute pancreatitis

Answer 64

Grey Turner sign

Question 65

Classically causes follicular keratoses, hemorrhage, and corkscrew hairs

Answer 65

Vitamin C deficiency

Question 66

Causes pellagra with its triad of diarrhea, dermatitis, and dementia.

Answer 66

Deficiency of vitamin B3

Question 67

Occurs in obstructive jaundice and leads to impaired coagulation, resulting in cutaneous hemorrhage with ecchymoses and purpura.

Answer 67

Malabsorption of vitamin K

Question 68

Presents with angular stomatitis, smooth painful tongue, and fragile/brittle nails, which have longitudinal ridging and lamellation. In severe deficiency, koilonychia, a spoonlike convexity of the nails, develops. Hair changes include diffuse scalp alopecia with brittle, split hairs. All take a long time to resolve on replacement therapy.

Answer 68

Iron deficiency

Question 69

Usually presents at weaning or in very early infancy with dermatitic or vesicobullous lesions of the hands, on the feet, and around the mouth and anus, together with progressive alopecia and “failure to thrive.”

Answer 69

Acrodermatitis enteropathica

Question 70

May appear in patients with chronic hepatitis, whatever the underlying cause and even before glucocorticoid therapy

Answer 70

Cutaneous striae

Question 71

Four main cutaneous associations of hepatitis B

Answer 71

1. A serum sickness-like syndrome
2. Cryoglobulinemia
3. Polyarteritis nodosa
4. Papular acrodermatitis of childhood

Question 72

Usually affects children. The appearance of monomorphic, flat-topped, erythematous papules on the face and limbs is characteristic. This eruption is self-limiting and asymptomatic in most, but may be accompanied by lymphadenopathy.

Answer 72

Papular acrodermatitis (Gianotti–Crosti syndrome)

Question 73

A rare autosomal recessive disorder characterized by ataxia, seizures, severe mental retardation, hepatomegaly with liver dysfunction, and brittle hair.

Answer 73

Argininosuccinic aciduria

Question 74

Occurs as a rare complication of Stevens–Johnson syndrome and has been reported to precede the skin signs.

Answer 74

Cholestatic hepatitis

Question 75

Two skin changes that occur in acute renal failure

Answer 75

Edema and uremic frost

Question 76

Vascular calcification is common in patients with long-term CRF, and is seldom symptomatic. Occasionally, however, calcified vessels may thrombose acutely, resulting in a this syndrome. This acute thrombosis produces livedoid areas that are excruciatingly painful due to ischemia, and quickly become hemorrhagic and ulcerate. PTH is usually markedly elevated.

Answer 76

Calciphylaxis

Question 77

Resembles scleromyxedema clinically and histopathologically. Largely occurs in patients with end stage renal disease, most of whom are on hemodialysis and described in a few patients with ARF. Clinically, patients progressively develop erythematous, sclerotic dermal plaques on the arms and legs, with sparing of the head and neck. Pruritus is a common feature.

Answer 77

Nephrogenic fibrosing dermopathy

Question 78

Involvement on the back of the hands over the knuckles and even on the palms can be seen. When the palms are involved, the rugated appearance of the palmar surface has been called this which is usually associated with acanthosis nigricans.

Answer 78

Tripe palms

Question 79

Presents as tightness and thickening of the skin and periarticular connective tissue of the fingers, resulting in a painless loss of joint mobility. Initial involvement of the distal interphalangeal joints of the fifth digit usually progresses proximally to involve all fingers. Larger joints of the elbow, knee, and foot may be affected. The actual joint space, however, remains uninvolved, so it is not a true arthropathy. This disorder is characterized by the “prayer sign,” which is an inability to approximate the palmar surfaces and interphalangeal joint spaces with the hands pressed together and fingers separated.

Answer 79

Diabetic Limited Joint Mobility

Question 80

Disease of long-standing diabetes associated with obesity. Presents with the insidious onset of painless, symmetric induration and thickening of the skin on the upper back and neck. Spreading to the face, shoulders, and anterior torso may occur. The skin retains a nonpitting, woody, peau d’orange quality. May experience decreased sensation to pain and light touch over the affected areas and difficulties with upper extremity and neck range of motion.

Answer 80

Sclerema diabeticorum

Question 81

Present clinically as 1- to 4-mm, reddish-yellow papules on the buttocks and extensor surfaces of the extremities. The lesions occur in crops and may coalesce into plaques over time. These are generally asymptomatic but there is often underlying severe hypertriglyceridemia (>1,000 mg/dL) and potentially undiagnosed diabetes. Histologic and biochemical studies show that lipoproteins (mainly chylomicrons) in the blood permeate cutaneous vessel walls and accumulate in macrophages in the dermis.

Answer 81

Eruptive xanthomas

Question 82

On funduscopic examination, appears as pale pink to white retinal arterioles and venules. Triglyceride levels above 4,000. The fundus may have a milky hue.

Answer 82

Lipemia retinalis

Question 83

Callus formation precedes necrosis and breakdown of tissue over bony prominences of feet, usually on great toe and sole, over first and/or second metacarpophalangeal joints. These are surrounded by a ring of callus and may extend to underlying joint and bone. Complications are soft tissue infection and osteomyelitis.

Answer 83

Diabetic ulcers

Question 84

Bacterial infections More Common or Severe in Diabetes

Answer 84

Invasive Group B Strep
Invasive Group A Strep
Malignant external otitis
Necrotizing fasciitis

Question 85

This disorder was named after characteristic histologic findings and was first described in patients with diabetes. Presents with one to several sharply demarcated yellow–brown plaques on the anterior pretibial region. The lesions have a violaceous, irregular border that may be raised and indurated. Initially, often presents as red–brown papules and nodules that may mimic sarcoid or granuloma annulare (GA). Over time, the lesions flatten, and a central yellow or orange area becomes atrophic, and commonly telangiectasias are visible, taking on the characteristic “glazed-porcelain” sheen. Aside from the shins, other sites of predilection include ankles, calves, thighs, and feet.

Answer 85

Necrobiosis lipoidica diabeticorum

Question 86

Atrophic skin lesions of the lower extremity, or shin spots seen in diabetics. Presents as small (<1 cm), atrophic, pink to brown, scar-like macules on the pretibial areas. The lesions are asymptomatic and clear within 1-2 years with slight residual atrophy or hypopigmentation. The appearance of new lesions gives the sense that the pigmentation and atrophy are persistent.

Answer 86

Diabetic dermopathy

Question 87

Comprise an overlapping group of disorders characterized by transepidermal elimination or “spitting” of altered dermal constituents. Included in this group are Kyrle disease, reactive perforating collagenosis, perforating folliculitis, and elastosis perforans serpiginosa. these lesions appear as pruritic, keratotic papules mainly on the extensor surfaces of the extremities.

Answer 87

Acquired perforating disorders

Question 88

Abrupt, spontaneous development of blisters on the lower extremities without other demonstrable cause usually the toes, feet, and shins, arising in normal skin. The pathogenesis is unknown. The blisters are usually painless and not pruritic. Healing occurs within 2-5 weeks and rarely leaves scarring. Histopathologic examination of the bullae shows an inconsistent level of separation varying from intraepidermal to subepidermal. No immunopathologic features are consistently observed.

Answer 88

Bullosis diabeticorum

Question 89

This hormone is secreted by adipose tissue in proportion to total body fat. It regulates energy homeostasis, neuroendocrine function, and metabolism. Rare cases in humans have shown that its deficiency causes extreme obesity, hyperphagia, diabetes, neuroendocrine abnormalities, and infertility, all of which can be reversed by administration of exogenous.

Answer 89

Leptin

Question 90

Is an endogenous hypothalamic melanocortin receptor antagonist, which causes obesity when overexpressed. The gene is closely related to agouti, a skin pigmentation gene, which causes yellow coat color and obesity when overexpressed in mice.

Answer 90

Agouti-related protein (AGRP)

Question 91

Definition of Metabolic syndrome

Answer 91

1. Abdominal Obesity
2. Serum triglycerides ≥150 mg/dl (or drug treatment for elevated triglycerides)
3. Serum HDL cholesterol <40 mg/dl in men and <50 mg/dl in women (or drug treatment for low HDL-C)
4. Blood pressure ≥130/85 (or drug treatment for elevated blood pressure)
5. Fasting plasma glucose ≥100 mg/dl (or drug treatment for elevated blood glucose)

Question 92

Refer to the hyperplastic growth of thyroid tissue that is functioning independent of TSH regulation, resulting in increased levels of thyroid hormone.

Answer 92

Toxic adenoma and toxic multinodular goiter

Question 93

Results from the excess levels of thyroid hormones with resultant hypermetabolism

Answer 93

Hyperthyroidism (also known as thyrotoxicosis)

Question 94

Is characterized by hypometabolism secondary to diminished levels of thyroid hormone.

Answer 94

Hypothyroidism (or myxedema)

Question 95

Hypertrophy of the thyroid gland from persistent stimulation

Answer 95

Goiter

Question 96

Cutaneous findings in Hyperthyroidism

Answer 96

Soft, velvety, infant-like skin 
Thyroid dermopathy 
Soft, fine with diffuse nonscarring alopecia 
Facial flushing 
Palmar erythema 
Hyperpigmentation 
Plummer’s nails 
Thyroid acropachy 
Hyperpigmentation

Question 97

Concave shape nails with distal onycholysis

Answer 97

Plummer’s nails

Question 98

Most commonly seen with Graves disease but has been reported in hypothyroid patients as well. Occurs bilaterally as painless nonpitting nodules and plaques with variable coloring and a waxy, indurated texture. The distribution can range from very circumscribed to diffuse, but by far the most common location is on the extensor surfaces of the legs. Some cases can exhibit a peau d’orange appearance as well.

Answer 98

Thyroid dermopathy

Question 99

Refers to digital clubbing, softtissue swelling of the hands and feet, and the presence of characteristic periosteal reactions. This very rare clinical finding is also associated with thyroid dermopathy and exophthalmos, and almost always presents after the diagnosis and treatment of hyperthyroidism.

Answer 99

Thyroid acropachy

Question 100

Cutaneous findings in Hypothyroidism

Answer 100

Myxedema 
Cool temperature 
Doughy, dry skin with fine wrinkling 
Yellow–orange carotenemia 
Dry, brittle hair 
Slow growth of hair and nails

Question 101

The most classic finding associated with hypothyroidism. It occurs as a result of dermal accumulation of mucopolysaccharides, namely, hyaluronic acid and chondroitin sulfate, and tends to resolve with treatment of the hypothyroidism. Tends to be generalized but can appear more striking in the extremities. Characteristic facial changes can be seen, including a broadened nose, thickened lips, puffy eyelids, and macroglossia with a smooth and clumsy tongue. The skin can be doughy, swollen, and waxy, but without pitting.

Answer 101

Myxedema

Question 102

Facial plethora suggestive of compression when patients with large retrosternal goiters raise their arms above their head.

Answer 102

Pemberton’s sign

Question 103

The most common cause of hypercalcemia in outpatients

Answer 103

Hyperparathyroidism

Question 104

Most common cause of hypercalcemia in hospitalized patients

Answer 104

Malignancy

Question 105

Most common causes of hypocalcemia

Answer 105

Vitamin D deficiency

Surgically induced hypoparathyroidism

Question 106

Secreted by the parathyroid gland in response to low levels of serum calcium and acts as one of the main regulators of calcium levels in the body.

Answer 106

Parathyroid hormone (PTH)

Question 107

Results from abnormal function of the parathyroid gland, resulting in increased levels of PTH.

Answer 107

Primary hyperparathyroidism

Question 108

Cutaneous findings in Hypoparathyroidism

Answer 108

Scaly, hyperkeratotic, puffy skin
Patchy alopecia with hair thinning
Brittle nails with transverse ridging

Question 109

Excessive production of endogenous cortisol manifests clinically as

Answer 109

Cushing syndrome

Question 110

Increased central adiposity (moon facies and buffalo hump) with thinning of the extremities
Skin thinning and easy bruisability
Violaceous striae
Acanthosis nigricans
Increased dermatophyte and candidal skin and nail infections
Rarely, hyperpigmentation

Answer 110

Cutaneous findings in Cushing syndrome

Question 111

Hyperpigmentation of skin and mucous membranes
Longitudinal pigmented bands in the nails
Vitiligo
Decreased axillary and pubic hair in women
Calcification of auricular cartilages in men

Answer 111

Cutaneous findings in Addison disease

Question 112

Destruction of the adrenal glands and the resulting life-threatening deficiency of glucocorticoids, mineralocorticoids, and adrenal androgens

Answer 112

Addison disease

Question 113

In the setting of an acute bacterial infection, bilateral adrenal infarction due to adrenal hemorrhage is known as

Answer 113

Waterhouse–Friderichsen syndrome

Question 114

Kligman’s formula

Answer 114

5% hydroquinone

0. 1% tretinoin
1. 1% dexamethasone

Question 115

Sudden onset of acne
Severe acne refractory to conventional treatments
Irregular menstrual periods
Hirsutism

Answer 115

Hyperandrogenism

Question 116

Increased central adiposity (moon facies and buffalo hump) with thinning of the extremities
Skin thinning and easy bruisability
Violaceous striae
Acanthosis nigricans
Increased dermatophyte and candidal skin and nail infections
Rarely, hyperpigmentation

Answer 116

Cutaneous findings in Cushing syndrome

Question 117

Secreted by the pituitary in a pulsatile fashion and reaches its maximum rate during puberty and then declines over time

Answer 117

Growth Hormone

Question 118

Enlargement of hands and feet with a doughy texture
Increased ring, glove, shoe, and hat size
Jaw enlargement with separation of teeth (prognathism)
Macroglossia
Cutis vertices gyri
Acanthosis nigricans
Hirsutism
Hyperhidrosis
Brittle thick nails

Answer 118

Cutaneous findings in Acromegaly

Question 119

A multisystem granulomatous disease of unknown cause. The lung is the most commonly affected organ, but the skin is frequently involved.

Answer 119

Sarcoidosis

Question 120

Development of sarcoidosis requires at least three major events:

Answer 120

1. Exposure to antigen(s),
2. Acquired cellular immunity directed against the antigen and mediated through antigen-presenting cells and antigen-specific T lymphocytes
3. The appearance of immune effector cells that promote a nonspecific inflammatory response

Question 121

Describes the relatively symmetric, violaceous, indurated plaques and nodules that occur on the nose, earlobes, cheeks, and digits. This clinical variant of sarcoidosis is distinctive and has been associated with systemic involvement.

Answer 121

Lupus pernio

Question 122

Pink papules and plaques with prominent telangiectasias that usually occur on the face and may be a variant of lupus pernio, as both lesions usually appear on the face and can be violaceous. However, these tend to be less numerous than the papular and lupus pernio forms.

Answer 122

Angiolupoid lesions

Question 123

Rarely, sarcoidosis can present as persistent subcutaneous nodules. The nodules may be tender or painless and preferentially occur on the extremities.

Answer 123

Darier-Roussy sarcoid

Question 124

The bilateral enlargement of the lacrimal, parotid, sublingual, and submandibular glands may be caused by Sarcoidosis

Answer 124

Mikulicz syndrome

Question 125

The main nonspecific cutaneous manifestation of sarcoidosis (prevalence of approximately 17%)

Answer 125

Erythema nodosum

Question 126

The most common organ involved with sarcoidosis

Answer 126

Lung

Question 127

The most common ocular manifestation in sarcoidosis and can lead to cataracts and glaucoma

Answer 127

Uveitis

Question 128

Reflect the total granuloma burden in sarcoidosis; useful to monitor the course of sarcoidosis.

Answer 128

Serum Angiotensin-Converting Enzyme (SACE) levels

Question 129

Histopathology: noncaseating granulomatous inflammation with multinucleated giant cells and a surrounding zone of lymphocytes.

Answer 129

Sarcoidosis

Question 130

Chest radiograph findings: sarcoidosis

Answer 130

Asymptomatic hilar adenopathy

Question 131

Bilateral lacrimal and parotid gland uptake in Gallium-67 scanning

Answer 131

Panda sign

Question 132

Bilateral hilar and right paratracheal uptake in Gallium-67 scanning

Answer 132

Lambda sign

Question 133

Good Prognosis for Sarcoidosis

Answer 133

Erythema nodosum

Stage I vs. II–III CXR

Question 134

Poor Prognosis for Sarcoidosis

Answer 134

African descent
Extrathoracic disease
Age >40 years
Splenic involvement
Lupus pernio
Disease duration >2 years
FVC <1.5 L
Stage IV CXR/aspergilloma