Nail clubbing

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Clubbing
Other namesDrumstick fingers, digital clubbing, watch-glass nails [1]
Acopaquia.jpg
Clubbing
Specialty Pulmonology

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 and lungs. [2] [3] When it occurs together with joint effusions, joint pains, and abnormal skin and bone growth it is known as hypertrophic osteoarthropathy. [4]

Nail (anatomy) hard projection of digit

A nail is a horn-like keratinous envelope covering the tips of the fingers and toes in most primates. Nails evolved from claws found in other animals. Fingernails and toenails are made of a tough protective protein called alpha-keratin which is found in the hooves, hair, claws and horns of vertebrates.

Joint effusion effusion of watery liquid into the cavity of a joint

A joint effusion is the presence of increased intra-articular fluid. It may affect any joint. Commonly it involves the knee.

Hypertrophic osteoarthropathy medical condition combining clubbing and periostitis of the small hand joints

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 painful, swollen joints and synovial villous proliferation are often seen. The condition may occur alone (primary), or it may be secondary to diseases like lung cancer. It is especially associated with non-small cell lung carcinoma. These patients often get clubbing and increased bone deposition on long bones. Their presenting symptoms are sometimes only clubbing and painful ankles.

Contents

Clubbing is associated with lung cancer, lung infections, interstitial lung disease, cystic fibrosis, or cardiovascular disease. [5] Clubbing may also run in families, [5] and occur unassociated with other medical problems. [6] [7]

Lung cancer cancer in the lung

Lung cancer, also known as lung carcinoma, is a malignant lung tumor characterized by uncontrolled cell growth in tissues of the lung. This growth can spread beyond the lung by the process of metastasis into nearby tissue or other parts of the body. Most cancers that start in the lung, known as primary lung cancers, are carcinomas. The two main types are small-cell lung carcinoma (SCLC) and non-small-cell lung carcinoma (NSCLC). The most common symptoms are coughing, weight loss, shortness of breath, and chest pains.

Interstitial lung disease group of lung diseases affecting the interstitium (the tissue and space around the air sacs of the lungs)

Interstitial lung disease (ILD), or diffuse parenchymal lung disease (DPLD), is a group of lung diseases affecting the interstitium (the tissue and space around the alveoli. It concerns alveolar epithelium, pulmonary capillary endothelium, basement membrane, and perivascular and perilymphatic tissues. It may occur when an injury to the lungs triggers an abnormal healing response. Ordinarily, the body generates just the right amount of tissue to repair damage, but in interstitial lung disease, the repair process goes awry and the tissue around the air sacs becomes scarred and thickened. This makes it more difficult for oxygen to pass into the bloodstream. The term ILD is used to distinguish these diseases from obstructive airways diseases.

Cystic fibrosis Autosomal recessive disease

Cystic fibrosis (CF) is a genetic disorder that affects mostly the lungs, but also the pancreas, liver, kidneys, and intestine. Long-term issues include difficulty breathing and coughing up mucus as a result of frequent lung infections. Other signs and symptoms may include sinus infections, poor growth, fatty stool, clubbing of the fingers and toes, and infertility in most males. Different people may have different degrees of symptoms.

The incidence of clubbing is unknown; it was present in about 1% of people admitted to an internal medicine unit of a hospital. [5] Clubbing has been recognized as a sign of disease since the time of Hippocrates. [5]

Internal medicine is the medical specialty dealing with the prevention, diagnosis, and treatment of adult diseases. Physicians specializing in internal medicine are called internists, or physicians in Commonwealth nations. Internists are skilled in the management of patients who have undifferentiated or multi-system disease processes. Internists care for hospitalized and ambulatory patients and may play a major role in teaching and research.

Hippocrates ancient Greek physician

Hippocrates of Kos, also known as Hippocrates II, was a Greek physician of the Age of Pericles, who is considered one of the most outstanding figures in the history of medicine. He is often referred to as the "Father of Medicine" in recognition of his lasting contributions to the field as the founder of the Hippocratic School of Medicine. This intellectual school revolutionized medicine in ancient Greece, establishing it as a discipline distinct from other fields with which it had traditionally been associated, thus establishing medicine as a profession.

Causes

Clubbing is associated with:

Idiopathic pulmonary fibrosis chronic, irreversible and ultimately fatal disease characterized by a progressive decline in lung function

Idiopathic pulmonary fibrosis (IPF) is a type of chronic scarring lung disease characterized by a progressive and irreversible decline in lung function. Symptoms typically include gradual onset of shortness of breath and a dry cough. Other changes may include feeling tired and abnormally large and dome shaped nails. Complications may include pulmonary hypertension, heart failure, pneumonia, or pulmonary embolism.

Tuberculosis Infectious disease caused by the bacterium Mycobacterium tuberculosis

Tuberculosis (TB) is an infectious disease usually caused by Mycobacterium tuberculosis (MTB) bacteria. Tuberculosis generally affects the lungs, but can also affect other parts of the body. Most infections do not have symptoms, in which case it is known as latent tuberculosis. About 10% of latent infections progress to active disease which, if left untreated, kills about half of those affected. The classic symptoms of active TB are a chronic cough with blood-containing mucus, fever, night sweats, and weight loss. It was historically called "consumption" due to the weight loss. Infection of other organs can cause a wide range of symptoms.

Lung abscess lung disease characterized by microbial infection which causes a type of liquefactive necrosis of the pulmonary tissue and formation of cavities containing necrotic debris or fluid

Lung abscess is a type of liquefactive necrosis of the lung tissue and formation of cavities containing necrotic debris or fluid caused by microbial infection.

Nail clubbing is not specific to chronic obstructive pulmonary disease (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. [12]

A congenital form has also been recognized. [13]

Hypertrophic pulmonary osteoarthropathy

Bone scan of a patient with HPOA Marie-Bamberger2.jpg
Bone scan of a patient with HPOA

A special form of clubbing is hypertrophic pulmonary osteoarthropathy (HPOA), known in continental Europe as Pierre Marie-Bamberger syndrome. This is the combination of clubbing and thickening of periosteum (connective tissue lining of the bones) and synovium (lining of joints), and is often initially diagnosed as arthritis. It is commonly associated with lung cancer.[ citation needed ]

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. [14]

Pathophysiology

The exact cause for sporadic clubbing is unknown. Theories as to its cause include:

Diagnosis

Clubbing of the fingernail: The red line shows the outline of a clubbed nail. Clubbing.svg
Clubbing of the fingernail: The red line shows the outline of a clubbed nail.

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, which may disclose associated features relevant to the underlying diagnosis. Additional studies such as a chest X-ray and a chest CT-scan may reveal otherwise asymptomatic cardiopulmonary disease. [12]

Stages

Clubbing is present in one of five stages: [12]

Schamroth's test or Schamroth's window test (originally demonstrated by South African cardiologist Leo Schamroth on himself) [16] 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.

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. [7]

History

At least since the time of Hippocrates, clubbing has been recognized as a sign of disease. [5] The phenomenon has been called "Hippocratic fingers".

See also

Related Research Articles

Pleural effusion accumulation of excess fluid in the pleural cavity

A pleural effusion is excess fluid that accumulates in the pleural cavity, the fluid-filled space that surrounds the lungs. This excess fluid can impair breathing by limiting the expansion of the lungs. Various kinds of pleural effusion, depending on the nature of the fluid and what caused its entry into the pleural space, are hydrothorax, hemothorax (blood), urinothorax (urine), chylothorax (chyle), or pyothorax (pus) commonly known as pleural empyema. In contrast, a pneumothorax is the accumulation of air in the pleural space, and is commonly called a "collapsed lung".

Megakaryocyte

A megakaryocyte is a large bone marrow cell with a lobated nucleus responsible for the production of blood thrombocytes (platelets), which are necessary for normal blood clotting. Megakaryocytes usually account for 1 out of 10,000 bone marrow cells in normal people, but can increase in number nearly 10-fold during the course of certain diseases. Owing to variations in combining forms and spelling, synonyms include megalokaryocyte and megacaryocyte.

Pulmonary hypertension hypertension characterized by an increase of blood pressure in the pulmonary artery, pulmonary vein or pulmonary capillaries

Pulmonary hypertension is a condition of increased blood pressure within the arteries of the lungs. Symptoms include shortness of breath, syncope, tiredness, chest pain, swelling of the legs, and a fast heartbeat. The condition may make it difficult to exercise. Onset is typically gradual.

Eisenmengers syndrome fetal heart defect

Eisenmenger's syndrome is defined as the process in which a long-standing left-to-right cardiac shunt caused by a congenital heart defect causes pulmonary hypertension and eventual reversal of the shunt into a cyanotic right-to-left shunt. Because of the advent of fetal screening with echocardiography early in life, the incidence of heart defects progressing to Eisenmenger's has decreased.

CREST syndrome syndrome characterized by calcinosis, Raynauds phenomeno, esophageal dysmotility, sclerodactyly and telangiectasia

CREST syndrome, also known as the limited cutaneous form of systemic sclerosis (lcSSc), is a multisystem connective tissue disorder. The acronym "CREST" refers to the five main features: calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia.

Pulmonary fibrosis human disease

Pulmonary fibrosis is a respiratory disease in which scars are formed in the lung tissues, leading to serious breathing problems. Scar formation, the accumulation of excess fibrous connective tissue, leads to thickening of the walls, and causes reduced oxygen supply in the blood. A consequence is a perpetual shortness of breath.

Carcinoid syndrome Human disease

Carcinoid syndrome is a paraneoplastic syndrome comprising the signs and symptoms that occur secondary to carcinoid tumors. The syndrome includes flushing and diarrhea, and less frequently, heart failure, vomiting and bronchoconstriction. It is caused by endogenous secretion of mainly serotonin and kallikrein.

Respiratory disease disease of the respiratory system

Respiratory disease, or lung disease, is a medical term that encompasses pathological conditions affecting the organs and tissues that make gas exchange difficult in air-breathing animals. They include conditions of the respiratory tract including the trachea, bronchi, bronchioles, alveoli, pleurae, pleural cavity, and the nerves and muscles of respiration. Respiratory diseases range from mild and self-limiting, such as the common cold, to life-threatening diseases such as bacterial pneumonia, pulmonary embolism, acute asthma and lung cancer.

Pachydermoperiostosis (PDP) is a rare genetic disorder that affects both bones and skin. Other names are idiopathic hypertrophic osteoarthropathy or Touraine-Solente-Golé syndrome. It is mainly characterized by pachydermia, periostosis and finger clubbing.

Hermansky–Pudlak syndrome rare disease

Heřmanský–Pudlák syndrome is an extremely rare autosomal recessive disorder which results in oculocutaneous albinism, bleeding problems due to a platelet abnormality, and storage of an abnormal fat-protein compound.

Hypertrophic osteopathy is a bone disease secondary to cancer in the lungs.

Gray platelet syndrome rare congenital autosomal recessive bleeding disorder caused by a reduction or absence of alpha-granules in blood platelets

Gray platelet syndrome (GPS), or platelet alpha-granule deficiency, is a rare congenital autosomal recessive bleeding disorder caused by a reduction or absence of alpha-granules in blood platelets, and the release of proteins normally contained in these granules into the marrow, causing myelofibrosis.

In medicine, hepatopulmonary syndrome is a syndrome of shortness of breath and hypoxemia caused by vasodilation in the lungs of patients with liver disease. Dyspnea and hypoxemia are worse in the upright position.

Cirrhosis Chronic disease of the liver, characterized by fibrosis

Cirrhosis, also known as liver cirrhosis or hepatic cirrhosis, is a condition in which the liver does not function properly due to long-term damage. This damage is characterized by the replacement of normal liver tissue by scar tissue. Typically, the disease develops slowly over months or years. Early on, there are often no symptoms. As the disease worsens, a person may become tired, weak, itchy, have swelling in the lower legs, develop yellow skin, bruise easily, have fluid build up in the abdomen, or develop spider-like blood vessels on the skin. The fluid build-up in the abdomen may become spontaneously infected. Other serious complications include hepatic encephalopathy, bleeding from dilated veins in the esophagus or dilated stomach veins, and liver cancer. Hepatic encephalopathy results in confusion and may lead to unconsciousness.

Combined pulmonary fibrosis and emphysema (CPFE), describes a medical syndrome involving both pulmonary fibrosis and emphysema. The combination is most commonly found in male smokers. Pulmonary function tests typically show preserved lung volume with very low transfer factor.

Nintedanib chemical compound

Nintedanib, marketed under the brand names Ofev and Vargatef, is an oral medication used for the treatment of idiopathic pulmonary fibrosis and along with other medications for some types of non-small-cell lung cancer.

References

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  2. Rutherford, JD (14 May 2013). "Digital clubbing". Circulation. 127 (19): 1997–9. doi:10.1161/circulationaha.112.000163. PMID   23671180.
  3. Freedberg, et al. (2003). Fitzpatrick's Dermatology in General Medicine. (6th ed.). McGraw-Hill. ISBN   0-07-138076-0. :656
  4. Krugh, M; Vaidya, PN (January 2019). "Osteoarthropathy Hypertrophic". PMID   31082012.Cite journal requires |journal= (help)
  5. 1 2 3 4 5 Burcovschii, S; Aboeed, A (January 2019). "Nail Clubbing". PMID   30969535.Cite journal requires |journal= (help)
  6. Schwatz, RA. "Clubbing of the Nails". Medscape. Medscape. Retrieved 14 August 2014.
  7. 1 2 Vandemergel X, Renneboog B (July 2008). "Prevalence, aetiologies and significance of clubbing in a department of general internal medicine". Eur. J. Intern. Med. 19 (5): 325–9. doi:10.1016/j.ejim.2007.05.015. PMID   18549933.
  8. Sridhar KS, Lobo CF, Altman RD (1998). "Digital clubbing and lung cancer". Chest. 114 (6): 1535–37. doi:10.1378/chest.114.6.1535. PMID   9872183. Archived from the original (PDF) on 2003-11-01.Cite uses deprecated parameter |deadurl= (help)
  9. Epstein O, Dick R, Sherlock S (1981). "Prospective study of periostitis and finger clubbing in primary biliary cirrhosis and other forms of chronic liver disease". Gut. 22 (3): 203–6. doi:10.1136/gut.22.3.203. PMC   1419499 . PMID   7227854.
  10. Naeije R (March 2003). "Hepatopulmonary syndrome and portopulmonary hypertension". Swiss Med Wkly. 133 (11–12): 163–9. PMID   12715285.
  11. "acropachy" . GPnotebook.
  12. 1 2 3 Myers KA, Farquhar DR (2001). "The rational clinical examination: does this patient have clubbing?". JAMA. 286 (3): 341–7. doi:10.1001/jama.286.3.341. PMID   11466101.
  13. Shah K, Ferrara TM, Jan A, Umair M, Irfanullah, Khan S, Ahmad W, Spritz RA (August 2017). "Homozygous SLCO2A1 translation initiation codon mutation in a Pakistani family with recessive isolated congenital nail clubbing". Br. J. Dermatol. 177 (2): 546–548. doi:10.1111/bjd.15094. PMID   27681482.
  14. 1 2 Uppal S, Diggle CP, Carr IM, et al. (June 2008). "Mutations in 15-hydroxyprostaglandin dehydrogenase cause primary hypertrophic osteoarthropathy". Nat. Genet. 40 (6): 789–93. doi:10.1038/ng.153. PMID   18500342.
  15. Dickinson, CJ; Martin, JF (19 December 1987). "Megakaryocytes and platelet clumps as the cause of finger clubbing". Lancet. 2 (8573): 1434–5. doi:10.1016/s0140-6736(87)91132-9. PMID   2891996.
  16. Schamroth L (February 1976). "Personal experience". S. Afr. Med. J. 50 (9): 297–300. PMID   1265563.
Classification
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