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1.skin manifes system (final & update)

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  • 1. DR. HAM NAZMUL AHASAN PROFESSOR DEPARTMENT OF MEDICINE KHULNA MEDICAL COLLEGE, KHULNA
  • 2. Macule  A. A macule is a flat, nonpalpable skin lesion, recognizable only by virtue of a different color from the surrounding skin. Macules come in a white, brown, blue, black, or yellow or red or purple (erythematous purpuric) verities  B. Vilitigo. Sharply demarcated white macules. A B
  • 3. Papule  A schematic diagram of a papule, a small, elevated skin lesion usually unde 0.5 cm in diameter. Palpules are about equal in diameter and depth. They may have accompanying surface changes such as scale.
  • 4. Nodule  A, schematic diagram of a nodule, an elevated lesion greater than 0.5 cm in both width and depth. Papules, if they are neoplastic, will grow to become nodules, and if left in place, lesion that are nodules can grow to become tumors.  B, sarcoidosis. This patient has an eryhtematous nodule along the nasal rim. There is no surface change, but there are telangiectasias within the lesion.  C, Histopathology of sarcoidosis reveals a noncaseating granuloma with multinucleated giant cells in the dermis. The epidermis is normal. B C A
  • 5. Plaque  A, schematic diagram of a plaque, an elevated, plateaulike lesion greater in its diameter than its depth. The surface may or may not have secondary change such as scale or crusting. Plaques can be manifestation of an inflammatory process or a neoplastic process. They may due to primary disease in the epidermis or dermis.  B, this patient with psoriasis vulgaris demostrates a characteristic scaly plaque.  C, Histopathologic changes of psoriasis demonstrate the thickened epidermis with excess stratum corneum that correlates well with the clinical changes seen in B. A B C
  • 6. Vesicle & Bulla  A,schematic diagram of lesions that contain clear fluid. Vesicles are small lesions (0.5 cm in diameter), whereas bullae are larger lesions this lesion represent fluid accumulation within the epidermis or at the dermal-epidermal junction.  B, this patient with herpes gestations demonstrates both vesicles and bullae. The lesions are grouped. One area shows a resolving blister and this represents a crusted erosion. Vasicles and bullae are dynamic lesion; they become pustules and/or erosions with time. A B
  • 7. Pustule  A, schematic diagram of a pustule, a fluid filled sack that contains cloudy or purulent material.  B, Pustular psoriasis is a rare and potentially life threatening manifestation of psoriasis characterized by sheet of small pustules on an erythematous base. A B
  • 8. Erosion, Ulcer & Fissure  A, schematic diagram demonstrates three changes. An erosin is a loss of surface epidermis that is superficial and often occurs after the natural breakage of a vesicle, bulla, or papule. An ulcer is a loss of epidermis that extends in to the dermis or subcutaneous areas and is usually as broad as it is deep. A fissure is a crack in the skin that is usually narrow but deep. Fissures and ulcer are often painful; erosions are merely uncomfortable.  B, pyoderma gangrenosum is a rare ulcerative disorder that is often a manifestation of a systemic disease. The epidermis is totally lost and, in this case the margin has cracks in the skin that represent fissures. A B
  • 9. Wheal  A, schematic diagram of wheal (hive). This represents variation on the papules or plaque in which the cause of lesion is dermal edema. These lesion have no epidermal change, are often transient, and may have a central pallor  B, in this patient with urticaria, it was possible to demonstrate the phenomenon of dermatographism, which occurs when wheals are induced after pressure. This example took place at the University of Michigan in a consenting patient on Friday before a home football game. A B
  • 10. CHRONIC ARSENICOSIS
  • 11. Chronic arsenicosis with wet gangrene
  • 12. Chronic arsenicosis with dry gangrene
  • 13. Chronic Arsenicosis with squamous cell carcinoma
  • 14. Chronic Arsenicosis with squamous cell carcinoma
  • 15. Complications of Chronic Arsenicosis  Total cases – 11  Skin manifestations – all  Systemic complications – 9 • Chronic renal failure – 3 • Pulmonary tuberculosis – 2 • Peripheral neuropathy – 2 • Neurogenic bladder – 1 • Chronic bronchiectasis – 1 • Hypoplastic anaemia – 1 • Malignancies – 4 (squamous cell Ca of skin – 3, bronchogenic carcinoma – 1) Nazmul Ahasan HAM. Complication of chronic arsenicosis. Tropical Doctor, 2001;31:28-29
  • 16. Rheumatologic Disease
  • 17. “Lupus erythemateux.” Lecons sur les Maladies de la peau. Pierre Louis Alphee Cazenava 1856
  • 18. Malar erythema Malar erythema, the so-called butterfly rash, is often abrupt in onset and frequently accompanies flares of active SLE. Nonscarring erythema and edema can last hours to days and occasionally longer. Malar areas are most typically affected, but diffuse facial involvement can occur. Erythema of sun-exposed and sun-protected areas of the trunk and extremities also occurs, often with exacerbation of systemic symptoms. Treatment of the systemic disease will result in the resolution of cutaneous symptoms.
  • 19. Discoid lupus erythematous Discoid lupus erythematous is most commonly seen in patients with a form of lupus that has been termed chronic cutaneous lupus erythematosus. Unlike malar erythema, which is associated with acute lupus erythematosus, patients with discoid lupus erythematosus usually have a more benign course that is not associated with the internal organ involvement of SLE. Lesions are typically eryhematous papules, patches, or plaques that show follicular plugging (i.e. dilated hair follicles filled with hyperkeratotic plugs). The patches and plaques are often round with a border of hyperpigmentation that can expand. Individual lesion can become crusted and heal with scarring and central depigmentation. Discoid lupus eryhtema generally affects the face, scalp, and ears. The term generalized discoid lupus eryhtematosus is used for patients who have cutaneous involvement of the trunk and extremities as well as involvement above the neck. It has been suggested that patients with generalized discoid lupus erythematous may be more prone to develop symptoms of SLE.
  • 20. Scarring Alopecia A scarring alopecia can occur as a result of discoid lupus erythematous
  • 21. Photosensitivity Photosensitivity, third cutaneous criterion for the diagnosis of SLE, has been shown to occur as a result of exposure to ultraviolet light. Patients must therefore be counseled on use of broad spectrum sunscreens as well as avoidance of sun exposure. The face, “V” of the neck and chest, and sun-exposed areas of the arms and legs are characteristically affected. Psoriasiform, erythematous, polycylic, and discoid lesion have been described
  • 22. Oral ulcers in SLE  Oral ulcer, constitute a fourth cutaneous criterion for the diagnosis of SLE. These oral or nasopharyngeal ulceration are typically painless, but in addition to ulcerations a variety of mucous membrane lesions, including petechiae, purpura, and ersions, have been described.
  • 23. SLE
  • 24. SLE with Addison’s disease
  • 25. Periungual Telangietasia Cutaneous symptoms are so intimately associated with rheumatic diseases that they appear prominently in the criteria for diagnosis of such disorders as lupus, scleroderma, and dermatomyositis. Several skin lesions such as periungual telangietasia are non-specifically associated with rheumatic diseases, although they also occur with other conditions or in the absence of systemic disease. Cuticular telangietasia is seen in lupus, dermatomyositis, and scleroderma and in a small proportion of rheuma.
  • 26. Raynoud’s Phenomenon Raynoud’s phenomenon is another condition commonly associated with rheumatic diseases. It is characterized by episodic vasospasm resulting in ischemia of the digits. Episodes are generally trigged by exposure to cold or emotional stress. The characteristic color changes of white, blue, and red respectively, represent initial pallor caused by arterial vasoconstriction, cyanosis due to stagnant blood flow, and erythema, which results from reactive hyperemia. The color changes are associated with pain, tingling, burning and occasionally swelling. Raynaud’s phenomenon is Firly common affecting up to 10 percent of otherwise healthy females; women are effected almost eight times more frequently than men. Over 90 percent of cases Raynaud’s phenomenon are not associated with an underlying cause. These patients have a fairly benign course and their condition is termed Raynaud’s disease. Raynaud’s phenomenon, however, can occur in conjunction with a number of disorders, particularly connective tissue diseases.
  • 27. Livedo Reticularis Livedo reticularis is a cutaneous pattern associated with connective tissue diseases. It is characterized by reticulated erythema, which is purplish at times, and most commonly affects the lower extremities, although the upper extremities and trunk can be affected.
  • 28. Recurrent Thrombophlebitis Recurrent thrombophlebitis affects some lupus patients, particularly those with false- positive serologic tests for syphilis and a circulating anticoagulant.
  • 29. Dermatomyositis  Dermatomyositis is an inflammatory process of striated muscle and skin that results in muscle weakness, particularly involving proximal muscles. In approximately 50 percent of cases, muscle involvement occurs in the absence of skin lesions, and the condition is then called polymyositis. Patient’s complaint include difficulty with brushing their hair and the inability to climb stairs or to rise from low seats. Muscle tenderness occasionally occurs.  In one third of patients, cutaneous symptoms precede muscle involvement. One of the most characteristic findings is periobital eryhtema and edema called heliotrope. This is often associated with a butterfly rash that simulates lupus.
  • 30. Dermatomyositis Gottron’s papules constitute a second characteristic sign of dermatomyositis. These consist of erythematous mascules and papules on the knuckles and, although they are said to be pathognomonic of dermatomyositis, they can occasionally be seen in other connective tissue diseases, including lupus. Dermatomyositis has long been considered a sign of underlying malignancy. Malignancies in adults with dermatomyositis occur in any where from 15 to 50 percent of reported patients
  • 31. Dermatomyositis  Patients with dermatomyositis will occasionally develop eryhtema with scaling, telangiectasia, and atrophy, primarily involving malar areas and the “V” of the neck and chest; the rash can be exarbated by sun exposure. Extensor surfaces of the extremities, including the elbows and knees, are also involved, and the torso can be affected as well. Other cutaneous signs of dermatomyositis include Raymound’sphenomenon, periungual telangiectasia and erythema, and rarely, sclerodactyly
  • 32. Cutaneous lesions in rheumatoid arthritis Cutaneous lesions are common in rheumatoid arthritis. Diagnosis of this disabling disease is established by a combination of criteria that include morning stiffness, joint tenderness or pain on motion, joint swelling, subcutanous nodules, radiographic changes typical of rheumatoid arthritis, positive rheumatoid factor, and characteristic synovial fluid and pathology. Rheumatoid nodules are present in 20 to 25 percent of patients and have been associated with seropositivity (rheumatoid factor)and more severe arthritis. Periarticular sites and areas subjected to mechanical pressure are the most commonly involved, especially the areas around the elbows.
  • 33. The extensor surface of the forearms, achilles tendon, metacare pophalangeal and interphalangeal joints, ischial tuberosities, and sacrum can also deelop rheumatoid nodules. The nodules are firm and range in size from 5 mm to several centimetres in diameter. They are usually asymptomatic but can occasionally become infected or break down. Rheumatoid nodules can be misdiagnosed as gouty tophi, xanthomata, epidermoid cysts, or enlarged lymph nodes
  • 34. Scleroderma Scleroderma also known as progressive systemic sclerosis, is a connective tissue disease characterized by cutaneous induration and thickening. Systemic sclerodema can be classified as two distinct entitis, namely the CREST syndrome and diffuse scleroderma. The CREST syndrome (Calcinosis, and Telangiectasia) is more slowly progressive anf therefore carries a more benign prognosis, it is ditinguished by the presence of anti-centromere antibodies. Although cases of scleroderma without skin involvement (“sclerodera sine scleroderma”) have been described. Cutaneous symptoms are so common that the American Rheumatism Association’s subcommittee for scleroderma criteria found that only one symptoms, proximal scleroderma, is necessary for the diagnosis of systemic sclerosis. Skin involvement often begins with an inflammatory stage consisting of erythema and edema of the hands and, occasionally, face. This progresses to severe induration of the skin of the digits (sclerodactyly)
  • 35. Scleroderma A. Involvement of the face and trunk can occur as well. Patients often present with typical facies, characterized by pursed lips and bound down skin at the nose that creates a beak-like appearance. B. Areas of hypo- and hyperpigmentation can also be seen A B
  • 36. Scleroderma with Raynaud’s Phenomenon  Raynaud’s phenomenon occurs in 95 percent of patients and may be so severe that ishemia and loss of digits can ossur.  Clacinosis cutis occur in 10 percent of patients and can result in ulceration or severe as a focus for infection.
  • 37. Morphea Localized forms of scleroderma known as morphea are not associated with systemic symptoms and are characterized by localized plaques of indurated skin often surrounded by a violaceous halo. A generalized form of morphea can occur but is distinguished from systemic by the absence of Raynaud’s phenomenon, sclerodactyly, and internal organ involvement.
  • 38. Mixed connective tissue disease Mixed connective tissue disease is a controversial entity that combines features of scleroderma, lupus, and dermatomyotisis. Raynaud’s phenomenon, arthralgias, persistent digital swelling resulting in “sausage fingers,” and sclerodactyly are typical feature. Esophageal hypomotility, proximal myositis, and pulmonary disease can occur. Malar erythema, lesions similar to those seen in suacute cutaneous lupus, and lesions resembling discoid lupus are occasionally seen in mixed connective tissue disease. This has led some to hypothesize that this disorder is merely a form of SLE, but the presence of high titer antinuclear ribonucleoprotien antibodies servers as a distingiushing marker for mixed connective tissue disease.
  • 39. Scleredema  Scleredema is a scleroderma-like condition that follows a viral or bacterial infection in some patients and is associated with diabetes mellitus in other patients. Cutaneous induration is systemetric and usually occurs on the back of the neck and the adjacent upper back.
  • 40. Behcet’s disease  Behcet’s disease consists of a triad of oral, genital, and ocular lesions. Individuals of Mediterrannean or Japanese origin appear to be affected more frequently, but the condition has been reported worldwide, Males outnumber females by as much as five to one, and the condition occurs most frequently in the third decade, although patients have been described from ages 6 to 72  Recurrent genital ulcers occur in approximately 80 percent of patients with Behcet’s disease and affect the vulva, vagina, penis, scrotum, and perianal area. They are more painful in males and can be asymptomatic in females. Genital ulcers occur less frequently than oral aphthae and heal with scarring. Urethitis does not occur.
  • 41. Behcet’s disease Mild nondeforming migratory arthritis occurs in approximately 50 percent of patients with Behcet’s disease. The picture shows acute monarthritis of the first digit in a patient with Behcet’s disease.
  • 42. Psoriasis Psoriasis is a common skin disorder. Several distinct cutaneous reaction patterns have been described. Plaque psoriasis is characterized by sharply demarcated erythematous scaling plaques. The elbows and knees are typically involved, as are the scalp, intergluteal cleft, palms, soles, and genitals. Nonetheless, any part of the body can be affected, and in some patients the condition covers large portions of the trunk and extremities
  • 43. Respiratory Diseases
  • 44. Illustrates professor Boeck’s article on “Sarkoid of the Skin in the Journal of Cutaneous and Genito-Urinary Diseases, 1899
  • 45. Clubbing Clubbing is a non-specific of pulmonary and mediastinal diseases. The distal digits are resulting in a bulbous appearance.
  • 46. Cyanosis Cyanosis is a bluish skin color associated with reduced oxygen saturation of the blood in cutaneous capillaries
  • 47. Erythema Nodosum The course of sarcoidosis can be highly variable. Approximately 20 to 40 per ht of patients abruptly develop constitutional symptoms such as fever and weight loss over a 1 to 2 week period. Chest X-ray reveal bilateral hilar adenopathy, and patients with this rapidly evolving form of sarcoid may present with erythema nodosum. The latter condition is characterized by bilateral deep, tender, red nodules on the shins. It has been suggested that the development of erythema nodosum in association with sarcoidosis connetes a good prognosis, since this patients usually have an acute, self-limited from of the disease. In the absence of other signs of sarcoidosis, erythema nodosum is a non-specific finding that occurs in association with neumerous underlying etiology
  • 48. Wegener’s Grnulomatosis Wegener’s grnulomatosis is a widespread granulomatous vasculitis that prominantly the tipper and lower respiratory tracts and the kidney. Upper respiratory tract symptoms include sinus pain and bloody or purulent nasal discharge. Nasal mucosal ulceration, nasal septal perforation, and saddle nose deformity occur. Cough, hemoptysis rhinorrhea, pleuritic chest pain, and dyspnea are common. On chest x-ray pulmonary nodules can be seen
  • 49. Ehlers-Danlos syndrome Ehlers-Danlos syndrome consists of a group of inherited disorders that are characterized by hyper extensibility of the skin and joints. Systemic complication are caused by fragility of blood vessels. Dissecting aneurysms of the aorta and other vessels can occur, as can gastrointestinal perforation and bleeding. Spontaneous rupture of major arteries can he catastrophic. Prolapse of the mitral valve occurs in -some forms of the Ehlers—Danlos syndrome. hyper extensibility of the skin is easily demonstrated by pulling on skin of the arms
  • 50. Yellow Nail Syndromes The yellow nail syndromes characterized by a triad of yellow nails. lymphedema, and pleural effusion. Thickening and yellow discoloration of the nails is the initial in more than one-third of patients and can affect both the fingernails and the toe nails. The rate of nail growth is slowed, and paronychial infection is common. Eventually, nail abnormalities develop in 90 percent of patients with this syndrome
  • 51. Cardiovascular Diseases
  • 52. Rheumatic Fever with Erythema Marginatum
  • 53. Tuberous xanthomas Accelerated atherosclerosis is strongly associated with several of the hyperlipoproteinemias. Xanthomas result from disorder of lipid metabolism that cause the deposition of lipids in skin, tendons, fascia and pariosteum. Tuberous xanthomas are among the most dramatic and appear as yellow or red nodules ranging from a few millimeters to 5 cm or more in diameter. They often occur over the extensor surfaces of joints such the elbow.
  • 54. Gastrointestinal Diseases
  • 55. Beu’s line of the nails A number of non-specific manifestation occasionally associated with gastrointestinal diseases. Beu’s line of the nails, for example, can occur weeks after episode of acute gastrointestinal bleeding, just as they can develop after any extreme physical stress. The patient in this picture develop parallel horizontal ridge of several finger nails after two episode of hematemesis and melena. These horizontal depression in the nail plate grew out over several months.
  • 56. Acrochordons Acrochordons, more commonly called skin tags, are common cutaneous lesion that have been associated with pregnancy and obesity. These skin-colored or pigmented papules are pedunculated and usually occur in intertriginous areas, including the neck, axillae, and medial aspect of the thigh. Several reports have correlated the presence of acrochordons with an increased incidence of adenomatous polyps of the colon, but this association is controversial.
  • 57. Plummer-Vinson syndrome The Plummer-Vinson syndrome is a condition characterized by dysphagia associated with iron deficiency anemia. Most of the cutaneous features of this disorder are manifestations of iron deficiency anemia, including koilonychia and glossitis (manifested by a red, shiny tongue with atrophy of the lingual papillae). Angular stomatitis (fissuring, of the oral commisures) also occur. The cutaneous lesion are reversible with iron supplementation, which correct the dysphagia as well. The condition usually affects middle- aged women, but it has been reported to occur as early as adolescence, as well as in older patients. Dysphagia has been attributed to a spasm that is caused by iron deficiency. If the iron deficiency is nor corrected, irreversible anatomic changes develop below the cricoid cartilage.
  • 58. Gardner’s Syndrome Gardner’s Syndrome is an autosomal dominantly inherited disorder characterized by the development of premalignant polyps of the colon and rectum. Malignant degeneration of colonic polyps occur in approximately 50 percent of patients. Cuteneous lesions include epidermal cyst (formerly called Sebaceous Cyst) that are present at birth or develop early in life. They can be large and are frequently on the face, but can be found on the trunk and extremities. The patient had multiple epidermal cyst.
  • 59. Peutz-Jeghers syndrome Peutz-Jeghers syndrome is another autosomal dominantly inherited condition associated with intestinal polyposis. Small intestinal polyps are characteristic, but some patient also develop large bowel and stomach polyps. Polyps of the bladder, bronchus, esophagus, mouth, maxillary sinus and nasal mucosa have been reported. Although the polyps are thought to be hamartomas, approximately 5 percent of patients with this syndrome develops gastrointestinal malignancies, most commonly in the small intestine. Ovarian tumours can occur as well Skin lesion are characterized by the development of the pigmented macules that most commonly involve the lips and oral mucosa.
  • 60. Pyoderma Gangrenosum Pyoderma Gangrenosum is perhaps the best known dermatosis specially associated with inflammatory bowel disease. Of the first patient described with pyoderma gangrenomus, four out of five had ulcerative colitis. Only a small minority of patient with ulcerative colitis ever develop pyoderma gangrenosum, however.
  • 61. Pyoderma Gangrenosum In some patient with ulcerative colitis, the skin lesions of pyoderma gangrenosum are closely linked to the severity of the bowel disease. In these patients, exarbation of bowel disease. In these patients, exacerbation of bowel symptoms is often associated with extension of existing lesions and development of new lesions. Linkage of pyoderma gangrenosum to the severity of ulcerative colitis has been so close in some patients that severe skin disease has been used to justify surgical removal of diseased bowel.
  • 62. Pyoderma gangrenosum Pyoderma gangrenosum is a marker for crohn’s disease. Occurring in anywhere from 0.5 to 20 percent of patients with this bowel disorder. Pyoderma gangrenosum occuring in patients with crohn’s disease is indistinguishable from that associated with ulcerative colitis.
  • 63. Pellagra Pellagra is a condition that result from a dietary deficiency of niacin. Pellagra usually accompanies protein malnutrition, since niacin can be synthesized from tryptophan. The disorder is seen in condition of severe malnutrition and, in Western nations, is usually seen in malnourished alcoholics. The clinical features of this disorder are summarized by the three D’s: dermatitis, diarrhea, and dementia. The dermatitis can be limited to areas of sun exposure, including the face, neck, and dorsa of the arms, as shown in patient reported in 1925. affected areas are sharply demarcated and can be brightly erythematous with subsequent scaling and hyperpigmentation. The demarcation can be so striking that the collaratee of dermatitis around the neck has been termed Casal’s neckalce. Mucous membranes are affected, with swelling and redness of the tongue, aphthous ulcers, and fissures at the oral commisures. Diarrhea occurs in 40 to 50 percent of patients and may be associated with steatorrhea. Mental depression and dementia follow. With institution of niacin therapy, symptoms rapidly improve.
  • 64. Carcinoid Tumors  A striking syndrome with a number of cutaneous manifestations has been associated with carcinoid tumors, more than 90 percent of which originate in the gastrointestinal tract. The carcinoid syndrome most commonly occurs when intestinal carcinoid spreads to the liver, but a variant of the syndrome can Occur in patients with bronchial carcinoids in the absence of hepatic metastases.  Episodic flushing is the earliest symptom in many patients, although one-third of patients do not experience flushing. Redness begins on the face but soon involves the upper extremities and chest, Episodes initially last for only minutes and can occur several times in the same day.
  • 65. Carcinoid Syndrome The vascular nature of flushing in the carcinoid syndrome is demonstrated in the picture simple pressure by the physician’s fingertips results in localized blanching on the chest of a patient with a carcinoid flush. In some patients flushing is associated with episodic tremulousness, abdominal pain and explosive diarrhea, and paroxysmal bronchospasm characterized by shortness of breath and wheezing. Flushing can he triggered by palpation of liver metasta or abdominal tumors, alcohol intake, exertion, anger, tension, a number of foods. Including cheese and salty bacon, and a number of medications, including epinephrine.
  • 66. Hereditary hemorrhagic telangiectasia Gastrointestinal bleeding is a cardinal feature of hereditary hemorrhagic telangiectasia, also called Osler-Weber-Rendu disease. This autosomal dominantly inherited condition often begins with repeated epistaxis during childhood. Telangiectases become apparent during the second and third decades and occur on the nasal mucosa and on the lips.
  • 67. Acute Hemorrhagic Pancreatitis In acute hemorrhagic pancreatitis, retroperitoneal blood can dissect along facial planes into the skin of the flanks, the abdominal wall, and around the umbilicus. Purpura around the umbilicus has been called Cullen’s sign. Purpura in the flank (Grey-Turner’s sign) and Cullen’s sign indicate the presence of a severe necrotizing pancreatitis. Patients are acutely ill. Shock results from extravasations of blood into the retroperitoneal space and release of kinins and proteolytic and lipolytic enzymes.
  • 68. Migratory thrombophlebitis Migratory thrombophlebitis has been associated with several malignancies but most notably with pancreatic cancer. The patient shown in picture has obvious swelling of the left leg due to phlebitis. He developed repeated bouts of phlebitis in the arms and legs followed by the new onset of diabetes mellitus and weight loss. While thronihophlebitis and diabetes have classically been described in pancreatic carcinoma, they are actually infrequent presenting signs of this malignancy
  • 69. Jaundice There are numerous cutaneous manifestations of liver disease, including both sp and nonspecific findings. Jaundice is most prominent in the various causes of obstructive liver disease, but it can also occur in acute hepatitis and other hepatic disorders and, to a much lesser extent, in the setting of hemolysis, Jaundice becomes clinically apparent when serum bilirubin levels, exceed 2,5 to 3.0 mg/d or 6.0 mg/dl in newborn infants This condition is characterized by yellow discoloration of the skin and mucous membranes.
  • 70. Jaundice When mild jaundice is easily overlooked, although it may be more obvious on examination of the ocular sclerae than of the skin. It can gradually increase over days to weeks before becoming obvious. When hyperbilirubinemia is due to excessive conjugated (“direct”) bilirubin as in obstructive liver disease, urine becomes dark and tea-colored. Stools take on a tail, clay- like color. This does not occur when bilirubin is unconjugated (“indirect”).)
  • 71. Gynecomastia It has been thought that patients with cirrhosis develop hyperestrogenism as a result of the liver’s inability to metabolize estrogens. Whether or not that mechanism is correct, patients develop feminizing signs such as loss of facial, axillary, and pubic hair, testicular atrophy, and gynecomastia. Palmar erythema and spider angiomas have also been attributed to the hyperestrogenism of chronic liver disease. The patient shown in picture developed progressive hepatic failure mid gynecomastia as a result of severe amoebic infection.
  • 72. Palmer Erythema
  • 73. Urticaria There are a number of cutaneous signs of viral hepatitis, and several dermatologic syndromes are associated with hepatitis B, including polyarteritis nodosa, essential mixed cryoglobulinemia, and erythema nodosum. A serum sickness-like prodrome occasionally precedes other signs of hepatitis B by approximately 2 weeks. Affected patients develop art urticaria-like rash associated with arthralgias, Less frequently. erythematous or purpuric eruptions have been described.
  • 74. Renal Diseases
  • 75. Uremic Frost In advance renal failure, urea reaches high concentrations in sweat and, on evaporation, leaves a white powder that has been called uremic frost.
  • 76. Ecchymoses in Renal Diseases The cutaneous stigmata of uremia occur late in the course of renal disease and range from subtle to severe. Patients frequently develop ecchymoses as a result of a coagulation defect cans Lb abnormal p katelet functi on Hyperpigmentation is common, and skin often has a yellow hue. Probably the most troublesome symptom of patients on dialysis is severe, unremitting pruritus. Examination of affected individuals reveals generalized excoriations with out primary skin lesions.
  • 77. Acquired ichthyosis A number of different ichthyoses have been associated with elevations of parathyroid hormone. Several of the reported patients have had secondary hyperparathyroidism. The patient shown in picture was on chronic dialysis for renal failure and developed secondary hyperparathyroidism. While her parathyroid hormone levels were becoming elevated, she began to notice progressively drier skin. Cutaneous examination revealed large geometrically shaped scales on the trunk and extremities, features of acquired ichthyosis.
  • 78. Polyarteritis nodosa Polyarteritis nodosa results from necrotizing inflammation of muscular arteries, including small arteries in the subcutaneous tissue. Damage to the arterial wall leads to the formation of aneurysms, especially in areas where the affected artery bifurcate Red tender subcutaneous nodules can develop along the course of affected arteries; these consist of thrombus-filled aneurysms. Nodules range from 0.5 to 2.0 cm and may be solitary or multiple. They most commonly occur on the lower extremities
  • 79. Polyarteritis nodosa The nodules may be pulsatile if the underlying aneurysm is not filled with thrombus. Progressive vessel occlusion results in cutaneous infarction and ulceration
  • 80. Henoch-Sehonlein purpura Henoch-Sehonlein purpura is a leukocytoclastic vasculitis that affects the skin, kidneys, joints, and gastrointestinal tract. This syndrome can occur in adults but typically affects children. The most characteristic skin lesions are the same as those occurring in other forms of cutaneous vasculitis, namely symmetric palpable purpura of the lower extremities Less commonly, purpura that is macular or palpable can occur on the upper extremities or trunk. Joint involvement is variable, and patients may have arthralgias or frank arthritis with swelling and pain of affected joints. Renal involvement is manifested by red cells and casts in the urinary sediment, but this seldom progresses to significant impairment of renal function. Gastrointestinal symptoms include hemotemesis, melena, and abdominal pain. Symptoms can mimic the abdominal pain of appendicitis, leading to necessary surgery.
  • 81. Hematologic Diseases
  • 82. Meyer-Betz first demonstrate the photosensitizing potential of porphyrins by injecting himself with hematoporphyrin and exposing his skin to sunlight. Marked swelling and eryhtema developed in sun-exposed skin.
  • 83. Purpura Defects of platelets and coagulation factors result in spontaneous bleeding into the skin or easy bruising following minor trauma. Abnormalities of coagulation can he inherited or acquired but, regardless of the etiology, bleeding into the skin results in purpura. These purple patches are demonstrated in Figure 6-3 in a patient with hemophilia A. This X-linked disorder affects 1:10,000 nialc.s and results from a defect of factor VT Bleeding cart occur hours or days after trauma, and symptoms are determined by the site of bleeding. Skin muscles, and joints are commonly affected.
  • 84. Petechiae Petechiae are pinpoint red or purple macules that occur at sites of bleeding capillaries in the skin. They develop in patients with abnormalities of platelet function or cutaneous vasculitis. Petechiae occur with or without purpura in patients on medications, such as aspirin, that interfere with platelet function; in patients with autoimmune conditions, such as idiopathic thrombocy. Lopenic purpura, that cause a reduction in circulating platelets; in patients with reduced numbers of platelets because of bone marrow destruction or hyperplenism; and in patients with disorders, such as von Willebrand’s disease or uremia, in which platelet function is abnormal.
  • 85. Disseminated intravascular coagulation Disseminated intravascular coagulation ( is a life-threatening syndrome of widespread thrombus deposition in the microvasculature and concomitant bleeding- This syndrome complicates numerous conditions, including bacterial sepsis, motastatic neoplasms, obstetric disasters such as abruptio placentae, fat embolism, and extensive tissue damage from trau or burns. The thrombotic component of DIC results in peripheral acrocyanosis and, in some eases, gangrene of the nose, digits, or genitalia. There is massive consumption of platelets, fibrinogen and clotting factors which eventually become depleted. This Leads to paradoxical bleeding in many organs including the skill and mucous membranes
  • 86. Sickle cell anemia Sickle cell anemia should be considered in any young black patient presenting with spontaneously appearing leg ulcers. The ulcers affect a high proportion of patients with homozygaus sickle cell disease and are particularly common in patients with severe anemia. Ulcers typically occur over the lateral or medial malleoli and have been attributed to vaso-occlusive phenomena lead ing to cutaneous ischemia. Ankle ulcerations rarely occur in patients with other congenital hemolytic anemias such as thalassenmia and congenital spherocytosis.
  • 87. Fanconi’s anemia Fanconi’s anemia is an autosomal recessively inherited condition associated with numerous anatomic malformations and a high incidence of malignancy. Patients have increased spontaneous chromosomal abnormalities, including gaps, breaks, and translocations. Before the age of 10, patients develop a hypocellular bone marrow, leading to thrombocytopenia. leukopenia, and anemia. Skin lesions occur in most patients and consist of a number of pigmentary abnormalities. Cafe-au-lait spots are reported in many patients and are usually present at birth.
  • 88. Pernicious anemia Pernicious anemia is an autoimmune disorder caused by atrophy of the gastric mucosa and a reduction in the secretion of intrinsic factor. This results in cobalamin deficiency. Nat surprisingly, pernicious anemia is associated with other autoimmune diseases, including vitiligo, which occurs in up to 10 percent of patients. Sharply demarcated areas of complete loss of pigment develop around the eyes, nose, mouth, ears, and on perianal and genital skin. Hands, feet, and extensor surfaces of the extremities are also typically affected, but vitiligo can occur anywhere on the skin, The patient in picture was being treated for vitiligo when the maciocytic anemia and vitamin B deficiency of pernicious anemia were detected.
  • 89. Porphyrias  The porphyrias are a group of disorder caused by defects in haemoglobin synthesis resulting in accumulation of porphyrins in body tissues and fluids. Cutaneous symptoms occur in most, hut not all of the porphyrias.  Porphyria culanea tarda is the commonest porphyria. It is caused by a decrease in uroporphyrinogen decarboxylase activity. There are two forms of porphyria cutanea tarda, a sporadic or acquired form and an autosomal dominantly inherited form with low penetrance. The sporadic form is precipitated by ingestion of alcohol, estrogens, iron, hexachlorohonzene, or polychlorinated hydrocarbons. In recent years, human irnn VI US infection has been associated with porphyria cu tarda, as has hepatitis C virus infection.  Clinical symptoms tisually begin in the third or fourth decade. There is marked skin fragility, especially on the dorsa of the hands. Minor trauma results in formation of vesicles and hullae, which form crusts, and eventually heal with hypopigmentation and scars.
  • 90. Amyloidosis Amyloidosis consists a group of disorders in which amyloid fibrils are deposit ed èxtacellularly in the skin or in other organs. Skin manifestations at most corn mon in primary systemic amyloidosis or in amyloid associated with multiple myelama. In the latter forms of amyloidosis, amyloid fibrils consist of inimunoglobulin light chain material. When this material infiltrntcs the dermis, the most characteristic skin lesions are waxy, shiny papules, which commonly develop on he eyelids.
  • 91. Endocrine Diseases
  • 92. Hypothyroidism The dermatologic manifestations of hypothyroidis arc often C deposition of mucopolysaccharides, which bind water, occurs, result ing in a puffs’ water-logged feeling. The accuniulation of water in the dermis results in nonpitting edema that is panicularly noticeable a’ound the eyes and in acra] areas. Reduced cutaneous blood flow leaves the skin cold and pale, and reduced activity of sweat and sebaceous glands results in dry, coarse, and, occa—sionally, pruritie skin. Dryness and scalin can be so st’*ing that ichthyosi changes occur in some patients.
  • 93. Hypothyroidism Reduced metabolism of β- carotene in the diet of some patients results in carotenimia with yellowing of the skin. The yellow discoloration is noticeable on the hands of a hypothyroid patieat in FigtLre 7-4, with the hand of a euthyroid individual shown for comparison.
  • 94. Hypothyroidism Hair loss is common and affects the entire hody shows diffuse lo of scalp hair in a woman with untreated hypothyroidism, The hair that remains is dull. coarse, and brittle, in part becanse of reduced sel,nm secretiow.
  • 95. Hypothyroidism Loss of the outer third of the eyebrows is characteristic of hypothyroidism. Some of the hair loss attributed to hypothyroidism can he reversed by the administration of thyroid replacement.
  • 96. Hypothoiroidism Thyroid replacement quickly reverses many of the cutaneous features of hypothyroidism.
  • 97. Pretibial myxedema Pretibial myxedema is one of the cardinal features of Graves’ disease.
  • 98. Hyperthyroidism Separation of the nail plate from the nail bed called onycholysis, occurs in more than I0 percent of hyperthyroid patients.
  • 99. Hyperthyroidism  Several common dermatoses are said to be increased in patients with hyper thyroidism. These include atopic dermatitis, chronic urticaria, and generalized pruritus. An increased incidence of vililigo has been noted in patients with  Graves’ disease.
  • 100. Cushing’s syndrome  Cushing’s syndrome is most commonly caused by exogenous administration of systemic steroids.
  • 101. Cushing’s syndrome  Fragility of blood vessels leads to large ecchymoses even following trivial trauma.
  • 102. Cushing’s syndrome  Formation of large purple striae is characteristic, and these can affect large areas of the trunk and extremities.
  • 103. Addison’s disease  Addison’s disease develops with destruction of the adrenal glands by any cause.
  • 104. Addison’s disease  Pigmentation of oral mucosa develops in spots rather than diffusely. The degree of hyperpigmentation is not related to the severity of other symptoms of Addison’s disease.
  • 105. Diabetes Mellitus  Diabetic dermopathy, also called “shin spots.” refers to depressed, atrophic,  pigmented macules and pathces that are commonly found on the anterior aspect of the lower legs of diabetic patients
  • 106. Diabetes Mellitus  Diabetic bullae are blisters that occur in patients with severe diabetes.
  • 107. Diabetes Mellitus  Waxy skin and stiff joints of diabetes has been described in patients with insulin-dependent diabetes.
  • 108. Diabetes Mellitus  Peripheral neuropathy due to severe diabetes leads to loss of sensation.
  • 109. Diabetes Mellitus  Necrobiosis lipoidica dial,eticorum is characterized by chronic, indolent, ftiptomatic lesions that alt usually found on [ anterior and lateral surfaces of the lower legs. Lesions may he solitary or multiple and involve one or both legs. Less commonly, they occur on the arms and trunk. They begin as erythe ‘natnus nodLiles with sharply circumscribed borders that slowly enlarge. The center of the lesion eventually becomes atrophic and yellow, even while there is an actively enlarging red border. Epidermis overlying this center becomes shiny and atrophic, and blood vessels can be seen through the thinned skin. hi some  patients the condition gradually progresses, involving the entire pretihial area. With time, the lesions of necrobiosis lipoidica diabeticorum can ulcerate, heconi ing painful and susceptible to infection. The vast majority of patients with nee,ohiosis lipoidica rliabeticorum have overt or latent diabetes. Even in those without evidence of diabetes, there is often a family history of the disorder, and these patients may eventually develop abnormal glucose tolerance. Only rarely does the condition develop in patients without any evidence of diabetes. Figure 7-27 shows early necrobiosis, diaheticoruin with shiny yellow atrophic changes of the pretibial skin.
  • 110. Diabetes Mellitus  Scleredema is a condition characterized by rock-hard induration of the skin. Lt usually begins on the back and posterior ncck.
  • 111. Erythrasma in Diabetes Mellitus  The prevalence of erythrasma is unquestionably increased in diabetic patients.
  • 112. Cutaneous candidal infections in Diabetes Mellitus  Cutaneous candidal infections are increased in hyperglycemic patients, hut respond well to control of blood sugar and are not increased in well- controlled diabetic patient5.
  • 113. Acanthosis Nigricans  Despite its well known association with malignancies, acanthosis nigricans more cummonly occurs in patients with a number of endocrinopathies.
  • 114. Polycystic ovary syndrome  Pocystic ovary syndrome, also called the Stein- Leventhal syndrome, consists of a constellation of clinical symptoms associated with androgen excess.
  • 115. Neurologic Diseases
  • 116. Neurofibromatosis  Type I neurofibromatosis also called von Recklinghausen’s disease, is a fairly common inherited disorder:
  • 117. Neurofibromatosis  Neurofibromas are soft, skin-colored nodules that can be flat or pedunculatcd
  • 118. Tuberous sclerosis  Tuberous sclerosis is an inherited syndrome with distinctive skin lesions and neurologic symptoms
  • 119. Shagreen patch  The shagreen patch another disthrtive feature of tuberous sclerosis, is a skin- colored plaque that consists of dermal connective tissue and develops early in childhood.
  • 120. Adenoma sebaceum  Adenoma sebaceum, the characteristic skin lesion of tuberous sclerosis, consists of angiofibromas of the cheeks and nasolabial folds.
  • 121. Infectious diseases
  • 122. Lupus Vulgaris
  • 123. Splinter hemorrhages in endocarditis  subacute and acute hactedal endocarilitis are associated with a wide array of cutaneous findings.
  • 124. Osler’s nodes  Osler’s nodes occur in approximately 5 percent of patients with endocarditis.
  • 125. Janeway lesions  Janeway lesions are nontender erythematous or purpuric macules or nodules of the palms and soles in patients with acute bacterial cndocarditis.
  • 126. Dengue syndrome
  • 127. Acute meningococcemia  Acute meningococcemia is characterized by the abrupt onset of lever, headache, and mental confusion, which can be followed rapidly by the development of a hemorrhagic rash, vomiting.
  • 128. Leprosy  Leprosy is a mycobacteterial infection that is common.
  • 129. Leprosy  The earliest cutaneous lesions of leprosy are small solitary or multiple, hypopigmented macules that are often not associated with any neurologic symptoms. Lesions can grow to cover large areas
  • 130. Leprosy  Lesions of tuberculoid leprosy are extremely variable in their appearance and extent. The classic tuberculoid lesion is a hypopigmented macule.
  • 131. Post kala-azar dermal leishmanisis (PKDL) Post Kala-azar dermal leishmaniasis (PKDL) in a patient who had been treated for visceral leishmaniasis 2 years earlier. The patient was completely cured by further chemotherapy.
  • 132. Secondary syphilis  Secondary syphilis begins approximately 8 weeks after the appearance of the chancre and occasionally the two may overlap. Skin lesions can be quite varied.  Involvement of the palms and soles is characteristic of secondary syphilis.
  • 133. Secondary syphilis  Occasionally lesions of secondary syphilis take on striking serpiginous or arcuate patterns.
  • 134. Secondary syphilis  Condyloma lata are moist papules covered with an exudate that is teeming with spirochetes. These lesions, have a predilection for the genitals and for intertriginous areas such as the perianal region.
  • 135. Secondary syphilis  Mucous patches, another lesion of secondary syphilis,are shown on the soft palate of the patient.
  • 136. Immunodeficiency Diseases
  • 137. AIDS  The patient shown has numerous cutaneous stigmiata of AIDS. The umbilicated white papules on and around the lips, on the nose, and on the right malar area are lesions of molluscum contagiosum The verrucous papule On the upper lip is a wart, and the purple patches on and beneath the lips are lesions of Kaposi sarcoma.)
  • 138. Herpes zoster  Typical-appearing herpes zoster, characterized by grouped vesicles in a dermatological distribution, can he the first sign of HIV infection.
  • 139. Neoplastic Diseases
  • 140. Skin Nodules in Lung Cancer  Lung cancers account for the largest number of cutaneous metastases.
  • 141. Skin nodules in Breast cancer  The scalp also has a higher incidence of metastasis from a number of tumors of the breast, lung, and genitourinary tract. The patient had a breast carcinoma that metastasized to the scalp. Tumors of the scalp may ulcerate, resulting in a scarring alopecia that has been called alopecia neoplastica.
  • 142. Drug induced
  • 143. Stevens Johnson Syndrome
  • 144. The “Tip of the iceberg” Spider naevi
  • 145. Skin Manifestations of Systemic Diseases Systemic diseases Spider naevi Cirrhotic liver
  • 146. Steps of diagnosis  Detail history  Careful physical examination • General examinations including skin • Systemic examinations  Investigations Final Pearls