133: Skin Manifestations of Internal Organ Disorders Flashcards
What are the characteristics of central cyanosis?
Purple-blue discoloration due to low arterial oxygen saturation caused by lung disease or shunt physiology. Visible in warm areas of the skin, such as the tongue, oral mucosa, and conjunctivae.
What are the characteristics of peripheral cyanosis?
Normal arterial oxygen saturation but reduced blood flow due to low-output cardiac failure or vasoconstriction. Seen in cooler areas like the nose, lips, earlobes, and fingertips.
What are the nail changes associated with cardiovascular disease?
Nail changes include clubbing (increased curvature of the nails) and Quincke pulsation (flushing of the nail beds synchronous with the heartbeat, indicative of aortic regurgitation).
What is the significance of the diagonal earlobe crease (Frank sign) in relation to coronary artery disease?
The diagonal earlobe crease has been controversially associated with an increased risk of coronary artery disease, particularly in older individuals.
What are the common manifestations of systemic lupus erythematosus (SLE) related to the cardiovascular system?
Common cardiovascular manifestations include pericarditis, myocarditis, and Libman-Sacks endocarditis.
What are the potential cardiovascular complications associated with systemic sclerosis?
Complications include pericarditis, small vessel disease causing patchy fibrosis in the myocardium, and increased risk of myocardial infarction.
What is cyanosis and what causes it?
Cyanosis is a purple-blue discoloration of the skin caused by an increase in the absolute amount of desaturated hemoglobin.
What are Osler nodes and how do they present?
Osler nodes are painful, tender lesions resulting from immune complex deposition, located distally on the digital tufts, presenting as erythematous or hemorrhagic macules, papules, or nodules.
What is digital clubbing and what does it indicate?
Digital clubbing is characterized by increased distal fingertip mass and changes in nail plate curvature, often indicating underlying pulmonary or cardiovascular disease.
What are the skin manifestations associated with autoimmune connective tissue diseases?
Skin manifestations can include rashes, lesions, and changes in pigmentation, often linked to conditions like systemic lupus erythematosus and systemic sclerosis.
What is the clinical significance of Quincke pulsation?
Quincke pulsation is the flushing of the nail beds synchronous with the heartbeat, indicative of aortic regurgitation.
What is the relationship between psoriasis treatment and myocardial infarction?
There have been reports of decreased rates of myocardial infarction after treatment with tumor necrosis factor inhibitors and methotrexate in psoriasis patients.
What is the hallmark finding in small vessel disease of the myocardium associated with systemic sclerosis?
The hallmark finding is patchy fibrosis of the myocardium.
What is the likely cause of neonatal lupus in a newborn?
Neonatal lupus is caused by transplacental transfer of maternal antibodies, most often anti-Ro/SSA.
What is the histological composition of the valve deposits in Libman-Sacks endocarditis?
The deposits consist of immune complexes, fibrin, platelet thrombi, and mononuclear cells.
What is the most common cardiac manifestation in patients with systemic lupus erythematosus (SLE)?
The most common cardiac manifestation is pericarditis.
What skin manifestations are associated with sarcoidosis?
Skin manifestations include noncaseating granulomas appearing as smooth papules, plaques, or nodules, and can also present as psoriasiform, ulcerative, or photodistributed lesions.
What are the pulmonary manifestations associated with systemic sclerosis?
Pulmonary manifestations include interstitial lung disease (ILD) and pulmonary arterial hypertension, with symptoms like dyspnea on exertion and a nonproductive cough.
What is aquagenic wrinkling of the palms also known as?
Aquagenic palmoplantar keratoderma.
What are the signs of jaundice related to liver dysfunction?
Jaundice is characterized by a generalized yellow discoloration of the skin, mucous membranes, and sclerae when bilirubin levels exceed 3 mg/dL.
What pigmentary changes can occur in patients with cirrhosis?
Patients may develop guttate hypomelanosis, diffuse muddy gray color, hyperpigmentation in primary biliary cirrhosis, and striking generalized metallic gray or bronze-brown color in hemochromatosis.
What is cholestatic pruritus and its common causes?
Cholestatic pruritus can be severe and is more common in conditions like primary biliary sclerosis, sclerosing cholangitis, and biliary obstruction, often linked to retained cutaneous bile acids.
What are the clinical implications of hemochromatosis?
Hemochromatosis leads to iron overload, hepatomegaly, transaminitis, cirrhosis, increased risk of hepatocellular carcinoma, and diffuse bronze hyperpigmentation.
What are the common skin findings associated with eosinophilic granulomatosis with polyangiitis?
Skin findings include palpable purpura, livedo reticularis, and urticarial lesions.
What is the most common pulmonary manifestation of rheumatoid arthritis?
Interstitial lung disease (ILD).
What are the vascular lesions commonly seen in the hepatobiliary system?
Common vascular lesions include telangiectasias, spider angiomas, and palmar erythema.
What nail changes are associated with liver disease?
Nail changes include clubbing, koilonychia, thickening and transverse white lines (Muehrcke nails), Terry nails, and blue discoloration of the lunulae in Wilson disease.
What are the two main presentations of pulmonary involvement in systemic sclerosis?
Interstitial lung disease (ILD) and pulmonary arterial hypertension.
What skin findings are associated with granulomatosis with polyangiitis?
Skin findings include palpable purpura, oral ulcers, and papulonecrotic lesions.
What is Yellow Nail Syndrome characterized by?
Thickened and yellow or yellow-green nails, disappearance of the cuticle, and associated nail abnormalities.
What are the clinical implications of hemochromatosis?
Hemochromatosis is caused by a mutation in the HFE gene, leading to increased intestinal iron absorption and iron overload. Clinical implications include: - Iron deposition in the liver causing hepatomegaly, transaminitis, and eventual cirrhosis. - Increased risk of hepatocellular carcinoma. - Diffuse bronze hyperpigmentation visible in 70% of patients at presentation due to iron deposition and increased melanin.
What is graft-versus-host disease and its common skin manifestations?
Graft-versus-host disease (GVHD) is a common complication of allogeneic hematopoietic stem cell transplant, where transplanted immune cells recognize the recipient’s cells as foreign. Common skin manifestations include: - Acute GVHD: nonspecific morbilliform eruption. - Chronic GVHD: poikiloderma, lichen planus-like lesions, and scleroderma-like lesions, often involving the eyes and oral mucosa.
What is a rare condition associated with cystic fibrosis that causes transient formation of papules and plaques on the palms?
Edematous, translucent papules and plaques that form within minutes of water exposure.
What infectious diseases may present with characteristic cutaneous findings?
Tuberculosis, endemic mycoses (e.g., coccidiomycosis, blastomycosis), and angioinvasive fungi (e.g., aspergillosis, mucormycosis).
What are spider angiomas and their significance in alcoholic cirrhosis?
Spider angiomas are small vascular lesions that can indicate esophageal varicosities and future risk of esophageal bleeding in alcoholic cirrhosis.
What nail changes are associated with iron deficiency anemia and hemochromatosis?
Koilonychia, which is a spoon-shaped nail deformity.
What is the bilirubin level that indicates jaundice?
Total serum bilirubin levels greater than 3 mg/dL.
What skin changes can occur in patients with long-standing cirrhosis?
Guttate hypomelanosis or diffuse muddy gray color, and hyperpigmentation in primary biliary cirrhosis.
What is cholestatic pruritus and its common causes?
Severe and refractory itching, more common in primary biliary sclerosis, sclerosing cholangitis, and biliary obstruction.
What are the signs of hemochromatosis?
Increased intestinal iron absorption, liver enlargement, transaminitis, and diffuse bronze hyperpigmentation.
What is graft-versus-host disease and its common manifestations?
A complication of allogeneic stem cell transplant where immune cells attack host cells, commonly affecting the skin, liver, and GI tract.
A patient with alcoholic cirrhosis develops spider angiomas on the face and upper chest. What is the significance of these lesions?
Spider angiomas are associated with esophageal varicosities and may be a predictive marker of future risk of esophageal bleeding.
What is Wilson Disease and what are its key features?
Wilson Disease is a hereditary condition characterized by defective cellular copper transport leading to copper accumulation in various organs, primarily the liver, brain, and cornea. Key features include: - Kayser-Fleischer rings: Corneal depositions visible with slit-lamp examination. - Symptoms: Can include liver dysfunction, neurological symptoms, and psychiatric issues.
What are the clinical manifestations of Primary Biliary Cirrhosis (PBC)?
Primary Biliary Cirrhosis (PBC) is an autoimmune disease affecting women aged 40 to 60, characterized by: - Destruction of intrahepatic bile ducts leading to hyperbilirubinemia and cirrhosis. - Symptoms: Pruritus, jaundice, diffuse hyperpigmentation, and xanthomas (which can be eruptive, planar, or tuberous). - Associated conditions: Lichen planus and potential association with limited scleroderma (CREST syndrome).
What skin manifestations are associated with Hepatitis B and C infections?
Hepatitis B and C infections can lead to various skin manifestations, including: - Transient rashes: Morbilliform, urticarial, or petechial. - Cryoglobulinemia: More common in HCV, often Type III, indicating active viral replication. - Gianotti-Crosti syndrome: Eruption of monomorphic, flat-topped, erythematous papules on the face and limbs, often seen in children.
What gastrointestinal conditions are associated with cutaneous findings?
Several gastrointestinal conditions are associated with cutaneous findings, including: - Hereditary hemorrhagic telangiectasia - Pseudoxanthoma elasticum - Ehlers-Danlos syndrome (vascular type) - Kaposi sarcoma - Mastocytosis - Inflammatory bowel disease - Polyposis syndromes. These conditions can lead to gastrointestinal bleeding and various skin manifestations.
What are the common skin manifestations of Inflammatory Bowel Disease (IBD)?
Inflammatory Bowel Disease (IBD) can present with several skin manifestations, including: - Recurrent episodes of abdominal pain - GI bleeding - Diarrhea - Skin lesions: Such as erythema nodosum and pyoderma gangrenosum, which may occur in association with IBD.
What are the key features of Dermatitis Herpetiformis?
Dermatitis Herpetiformis is a skin condition associated with celiac disease characterized by: - Intensely pruritic rash: Grouped excoriated papules and vesicles. - Location: Typically found over the buttocks and extensor surfaces. - Pathophysiology: Antitransglutaminase antibodies bind to keratinocytes, leading to the skin manifestations.
What is primary biliary cirrhosis and its common symptoms?
Primary biliary cirrhosis is an autoimmune disease affecting women aged 40 to 60, characterized by the destruction of intrahepatic bile ducts, leading to hyperbilirubinemia and cirrhosis. Common symptoms include pruritus, jaundice, diffuse hyperpigmentation, and xanthomas.
What skin condition is associated with hepatitis B and C infections?
Gianotti-Crosti syndrome, which presents as an eruption of monomorphic, flat-topped, erythematous papules on the face and limbs, is associated with hepatitis B and C infections.
What are some gastrointestinal diseases associated with GI bleeding and their cutaneous findings?
Diseases such as hereditary hemorrhagic telangiectasia, pseudoxanthoma elasticum, Ehlers-Danlos syndrome, Kaposi sarcoma, and inflammatory bowel disease are associated with GI bleeding and have cutaneous findings.
What are the common skin manifestations of malabsorption?
Common skin manifestations of malabsorption include xerosis, eczematous dermatitis, cheilitis, alopecia, changes in hair texture, nail changes, and hyperpigmentation.
What is the significance of Cullen sign and Grey Turner sign in abdominal pain?
Cullen sign (bruising around the umbilicus) and Grey Turner sign (bruising on the flanks) are well-known features of acute pancreatitis, indicating potential internal bleeding or severe abdominal conditions.
What is the relationship between dermatitis herpetiformis and celiac disease?
Dermatitis herpetiformis occurs in celiac disease when antitransglutaminase antibodies bind to keratinocytes, presenting as intensely pruritic grouped excoriated papules and vesicles over the buttocks and extensor surfaces.
What are the common etiologies of esophageal involvement in skin conditions?
Common etiologies include blistering dermatoses such as epidermolysis bullosa and cicatricial pemphigoid, as well as rheumatologic disorders like Behçet syndrome and systemic sclerosis (SSc).
What are the common organs involved in Kaposi Sarcoma?
The most common organs involved in Kaposi Sarcoma are the gastrointestinal tract and lungs.
A patient with primary biliary cirrhosis (PBC) presents with severe pruritus and hyperpigmentation. What is the underlying cause of these symptoms?
Pruritus is due to retained cutaneous bile acids, and hyperpigmentation is caused by melanin deposition.
A patient with hepatitis C virus (HCV) infection develops recurrent palpable purpura and arthritis. What is the likely underlying condition?
The likely condition is mixed cryoglobulinemia, which is usually Type III in HCV infection and may indicate active viral replication.
A patient with Wilson disease presents with brown corneal depositions visible on slit-lamp examination. What are these called, and what is their significance?
These are Kayser-Fleischer rings, which indicate copper deposition in the cornea due to defective cellular copper transport.
A patient with Ehlers-Danlos syndrome develops gastrointestinal bleeding. What is the likely cause?
The likely cause is vascular fragility, which can lead to bleeding in the gut.
A patient with chronic pancreatitis develops tender subcutaneous nodular areas of fat necrosis. What is the diagnosis?
The diagnosis is pancreatic panniculitis.
A patient with systemic sclerosis develops sclerodactyly and telangiectasia. What syndrome might this indicate?
This might indicate CREST syndrome, which includes calcinosis cutis, Raynaud phenomenon, esophageal motility disorder, sclerodactyly, and telangiectasia.
