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Nail clubbing, also known as digital clubbing or clubbing, is a deformity of the finger or toe nails associated with a number of diseases, mostly of the
heart The heart is a muscular organ in most animals. This organ pumps blood through the blood vessels of the circulatory system. The pumped blood carries oxygen and nutrients to the body, while carrying metabolic waste such as carbon dioxide to ...
and lungs.Freedberg, et al. (2003). ''Fitzpatrick's Dermatology in General Medicine''. (6th ed.). McGraw-Hill. . When it occurs together with
joint effusion A joint effusion is the presence of increased intra-articular fluid. It may affect any joint. Commonly it involves the knee. Diagnostic approach The approach to diagnosis depends on the joint involved. While aspiration of the joint is conside ...
s, joint pains, and abnormal skin and bone growth it is known as
hypertrophic osteoarthropathy Hypertrophic osteoarthropathy is a medical condition combining clubbing and periostitis of the small hand joints, especially the distal interphalangeal joints and the metacarpophalangeal joints. Distal expansion of the long bones as well as painf ...
. Clubbing is associated with lung cancer, lung infections, interstitial lung disease, cystic fibrosis, or
cardiovascular disease Cardiovascular disease (CVD) is a class of diseases that involve the heart or blood vessels. CVD includes coronary artery diseases (CAD) such as angina and myocardial infarction (commonly known as a heart attack). Other CVDs include stroke, h ...
. Clubbing may also run in families, and occur unassociated with other medical problems. The incidence of clubbing is unknown; it was present in about 1% of people admitted to an internal medicine unit of a hospital. Clubbing has been recognized as a sign of disease since the time of Hippocrates.


Causes

Clubbing is associated with * Lung disease: ** Lung cancer ** Interstitial lung disease most commonly idiopathic pulmonary fibrosis ** Complicated tuberculosis ** Suppurative lung disease: lung abscess,
empyema An empyema () is a collection or gathering of pus within a naturally existing anatomical cavity. For example, pleural empyema is empyema of the pleural cavity. It must be differentiated from an abscess, which is a collection of pus in a newly form ...
,
bronchiectasis Bronchiectasis is a disease in which there is permanent enlargement of parts of the airways of the lung. Symptoms typically include a chronic cough with mucus production. Other symptoms include shortness of breath, coughing up blood, and chest ...
, cystic fibrosis ** Mesothelioma of the pleura ** Arteriovenous fistula or malformation **
Sarcoidosis Sarcoidosis (also known as ''Besnier-Boeck-Schaumann disease'') is a disease involving abnormal collections of inflammatory cells that form lumps known as granulomata. The disease usually begins in the lungs, skin, or lymph nodes. Less commonly a ...
* Heart disease: ** Any disease featuring chronic hypoxia ** Congenital
cyanotic heart disease A cyanotic heart defect is any congenital heart defect (CHD) that occurs due to deoxygenated blood bypassing the lungs and entering the systemic circulation, or a mixture of oxygenated and unoxygenated blood entering the systemic circulation. It is ...
(most common cardiac cause) **
Subacute bacterial endocarditis Subacute bacterial endocarditis, abbreviated SBE, is a type of endocarditis (more specifically, infective endocarditis). Subacute bacterial endocarditis can be considered a form of type III hypersensitivity. Signs and symptoms Among the signs of ...
**
Atrial myxoma A myxoma is a rare benign tumor of the heart. Myxomata are the most common primary cardiac tumor in adults, and are most commonly found within the left atrium near the valve of the fossa ovalis. Myxomata may also develop in the other heart chambe ...
(benign tumor) ** Tetralogy of Fallot * Gastrointestinal and hepatobiliary: ** Malabsorption **
Crohn's disease Crohn's disease is a type of inflammatory bowel disease (IBD) that may affect any segment of the gastrointestinal tract. Symptoms often include abdominal pain, diarrhea (which may be bloody if inflammation is severe), fever, abdominal distension, ...
and ulcerative colitis **
Cirrhosis Cirrhosis, also known as liver cirrhosis or hepatic cirrhosis, and end-stage liver disease, is the impaired liver function caused by the formation of scar tissue known as fibrosis due to damage caused by liver disease. Damage causes tissue repai ...
, especially in
primary biliary cholangitis Primary biliary cholangitis (PBC), previously known as primary biliary cirrhosis, is an autoimmune disease of the liver. It results from a slow, progressive destruction of the small bile ducts of the liver, causing bile and other toxins to build ...
**
Hepatopulmonary syndrome In medicine, hepatopulmonary syndrome is a syndrome of shortness of breath and hypoxemia (low oxygen levels in the blood of the arteries) caused by vasodilation (broadening of the blood vessels) in the lungs of patients with liver disease. Dyspnea ...
, a complication of cirrhosis * Others: **
Graves' disease Graves' disease (german: Morbus Basedow), also known as toxic diffuse goiter, is an autoimmune disease that affects the thyroid. It frequently results in and is the most common cause of hyperthyroidism. It also often results in an enlarged thyro ...
(autoimmune hyperthyroidism) – in this case it is known as ''thyroid
acropachy Acropachy is a dermopathy associated with Graves' disease. It is characterized by soft-tissue swelling of the hands and clubbing of the fingers. Radiographic imaging of affected extremities typically demonstrates periostitis, most commonly the ...
'' ** Familial and hereditary clubbing and "pseudoclubbing" (people of African descent often have what appears to be clubbing) ** Vascular anomalies of the affected arm such as an
axillary artery aneurysm Axillary means "related to the axilla (armpit)" or "related to the leaf axils". "Axillary" may refer to: Biology * Axillary artery * Axillary border * Axillary fascia * Axillary feathers * Axillary hairs * Axillary lines * Axillary lymph node ...
(in unilateral clubbing) Nail clubbing is not specific to
chronic obstructive pulmonary disease Chronic obstructive pulmonary disease (COPD) is a type of progressive lung disease characterized by long-term respiratory symptoms and airflow limitation. The main symptoms include shortness of breath and a cough, which may or may not produce m ...
(COPD). Therefore, in patients with COPD and significant degrees of clubbing, a search for signs of bronchogenic carcinoma (or other causes of clubbing) might still be indicated. A congenital form has also been recognized.


Hypertrophic pulmonary osteoarthropathy

A special form of clubbing is
hypertrophic pulmonary osteoarthropathy Hypertrophic osteoarthropathy is a medical condition combining clubbing and periostitis of the small hand joints, especially the distal interphalangeal joints and the metacarpophalangeal joints. Distal expansion of the long bones as well as painf ...
(HPOA), known in continental Europe as Pierre Marie-Bamberger syndrome. This is the combination of clubbing and thickening of
periosteum The periosteum is a membrane that covers the outer surface of all bones, except at the articular surfaces (i.e. the parts within a joint space) of long bones. Endosteum lines the inner surface of the medullary cavity of all long bones. Structu ...
(connective tissue lining of the bones) and
synovium The synovial membrane (also known as the synovial stratum, synovium or stratum synoviale) is a specialized connective tissue that lines the inner surface of capsules of synovial joints and tendon sheath. It makes direct contact with the fibro ...
(lining of joints), and is often initially diagnosed as
arthritis Arthritis is a term often used to mean any disorder that affects joints. Symptoms generally include joint pain and stiffness. Other symptoms may include redness, warmth, swelling, and decreased range of motion of the affected joints. In some ...
. It is commonly associated with lung cancer.


Primary hypertrophic osteoarthropathy

Primary hypertrophic osteoarthropathy is HPOA without signs of pulmonary disease. This form has a hereditary component, although subtle cardiac abnormalities can occasionally be found. It is known eponymously as the Touraine–Solente–Golé syndrome. This condition has been linked to mutations in the gene on the fourth chromosome (4q33-q34) coding for the enzyme 15-hydroxyprostaglandin dehydrogenase (HPGD); this leads to decreased breakdown of prostaglandin E2 and elevated levels of this substance.


Pathogenesis

The exact cause for sporadic clubbing is unknown. Theories as to its cause include: *
Vasodilation Vasodilation is the widening of blood vessels. It results from relaxation of smooth muscle cells within the vessel walls, in particular in the large veins, large arteries, and smaller arterioles. The process is the opposite of vasoconstriction ...
(i.e., distended blood vessels). * Secretion of growth factors (e.g., platelet-derived growth factor and
hepatocyte growth factor Hepatocyte growth factor (HGF) or scatter factor (SF) is a paracrine cellular growth, motility and morphogenic factor. It is secreted by mesenchymal cells and targets and acts primarily upon epithelial cells and endothelial cells, but also acts o ...
) from the lungs. * Overproduction of prostaglandin E2 by other tissues. * Increased entry of megakaryocytes into the systemic circulation. Under normal circumstances in healthy individuals, megakaryocytes that arise from the bone marrow are trapped in the pulmonary capillary bed and broken down before they enter the systemic circulation. It is thought that in disorders where there is right-to-left shunting or lung malignancy, the megakaryocytes can bypass the breakdown within the pulmonary circulation and enter the systemic circulation. They are then trapped within the capillary beds within the extremities, such as the digits, and release platelet-derived growth factor (PDGF) and
vascular endothelial growth factor Vascular endothelial growth factor (VEGF, ), originally known as vascular permeability factor (VPF), is a signal protein produced by many cells that stimulates the formation of blood vessels. To be specific, VEGF is a sub-family of growth factors ...
(VEGF). PDGF and VEGF have growth promoting properties and cause connective tissue hypertrophy and capillary permeability.


Diagnosis

When clubbing is observed, pseudoclubbing should be excluded before making the diagnosis. Associated conditions may be identified by taking a detailed medical history—particular attention is paid to lung, heart, and gastrointestinal conditions—and conducting a thorough
clinical examination In a physical examination, medical examination, or clinical examination, a medical practitioner examines a patient for any possible medical signs or symptoms of a medical condition. It generally consists of a series of questions about the pat ...
, which may disclose associated features relevant to the underlying diagnosis. Additional studies such as a
chest X-ray A chest radiograph, called a chest X-ray (CXR), or chest film, is a projection radiograph of the chest used to diagnose conditions affecting the chest, its contents, and nearby structures. Chest radiographs are the most common film taken in me ...
and a chest CT-scan may reveal otherwise asymptomatic cardiopulmonary disease.


Stages

Clubbing is present in one of five stages: * No visible clubbing - Fluctuation (increased ballotability) and softening of the nail bed only. No visible changes of nails. * Mild clubbing - Loss of the normal <165° angle (Lovibond angle) between the nailbed and the fold (cuticula). Schamroth's window (see below) is obliterated. Clubbing is not obvious at a glance. * Moderate clubbing - Increased convexity of the nail fold. Clubbing is apparent at a glance. * Gross clubbing - Thickening of the whole
distal Standard anatomical terms of location are used to unambiguously describe the anatomy of animals, including humans. The terms, typically derived from Latin or Greek roots, describe something in its standard anatomical position. This position prov ...
(end part of the) finger (resembling a drumstick) *
Hypertrophic osteoarthropathy Hypertrophic osteoarthropathy is a medical condition combining clubbing and periostitis of the small hand joints, especially the distal interphalangeal joints and the metacarpophalangeal joints. Distal expansion of the long bones as well as painf ...
- Shiny aspect and
striation Striations means a series of ridges, furrows or linear marks, and is used in several ways: * Glacial striation * Striation (fatigue), in material * Striation (geology), a ''striation'' as a result of a geological fault * Striation Valley, in Ant ...
of the nail and skin Schamroth's sign or Schamroth's window test (originally demonstrated by South African cardiologist
Leo Schamroth Leo Schamroth (2 June 1924 in Belgium – 24 May 1988 in Johannesburg) was a South African cardiologist remembered for his work in electrocardiography and for describing Schamroth's window test. Biography Schamroth was born in Belgium a ...
on himself) is a popular test for clubbing. When the distal phalanges (bones nearest the fingertips) of corresponding fingers of opposite hands are directly opposed (place fingernails of same finger on opposite hands against each other, nail to nail), a small diamond-shaped "window" is normally apparent between the nailbeds. If this window is obliterated, the test is positive and clubbing is present. File:Clubbing of fingers.jpg, Severe clubbing Clubbing2.JPG, Front view Clubbing1.JPG, Side views CongenitalHeartCase-133.jpg, Cyanotic nail beds


Epidemiology

The exact frequency of clubbing in the population is not known. A 2008 study found clubbing in 1%, or 15 patients, of 1511 patients admitted to a department of internal medicine in Belgium. Of these, 40%, or 6 patients, turned out to have significant underlying disease of various causes, while 60%, or 9 patients, had no medical problems on further investigations and remained well over the subsequent year.


History

At least since the time of Hippocrates, clubbing has been recognized as a sign of disease. The phenomenon has been called "Hippocratic fingers".


See also

*
Clubbed thumb Clubbed Thumb is a downtown theater company in New York City that commissions, develops, and produces "funny, strange, and provocative new plays by living American writers." Since its founding in 1996, the company has earned five OBIES (including t ...
(unrelated congenital deformity)


References

{{Respiratory system symptoms and signs Conditions of the skin appendages Symptoms and signs: musculoskeletal system