Shell nail syndrome | |
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Specialty | Dermatology, pulmonology |
Symptoms | clubbed fingernails |
Causes | bronchiectasis, hereditary factors, cardiopulmonary disorders, gastrointestinal disorders, onycholysis |
Diagnostic method | X-ray |
Differential diagnosis | clubbed fingernails, Crohn's disease, Hapalonychia |
Shell nail syndrome is a medical condition defined by the concurrence of large, rounded fingernails and bronchiectasis. [1] Despite the visual similarity between the two conditions, shell nail syndrome and clubbed fingernails are opposites. Shell nail syndrome results from atrophy to the nail bed, whereas clubbed fingernails results from a bulbous, hypertrophic growth of soft tissue. The concurrence of the syndrome and bronchiectasis is well-established, [2] [3] however the exact causes of the deformity remains unknown. [4] The syndrome has been observed affecting both the hands and larger toenails. [2]
The link between shell nail syndrome and bronchiectasis is present within the first description of the syndrome. The patient, a 37-year-old-woman admitted to hospital, recounts first noticing a "persistent dystrophy" of her fingernails at age five, this occurring one year after developing severe whooping cough followed by pneumonia. [2] A 2013 Clinics in Dermatology publication defines the disease as "nails [which] resemble clubbed nails with the exception of the distal nail bed, which is atrophic rather than hypertrophied and bulbous." [1]
At present, the specific cause of shell nail syndrome is unknown. American doctors Chalmers E. Cornelius and Walter B. Shelley first characterized the morphogenesis of the condition in a 1967 Archives of Dermatology publication, stating: "this disorder can be explained by the production of a curved nail by a normal nail matrix which grows out as a shell to thus form a roof over the atrophic process of the more acral portions of the digit." [2] This description of how the nail "grows out as a shell" [2] to cover the atrophied nail bed is where the syndrome takes its name. Diagnosis of shell nail syndrome is performed by x-ray of the patient's distal phalanges. A side view of a fingertip affected by shell nail syndrome will reveal an air space between the nail and nail bed. [2] The first description of the syndrome contains diagnosis by removal of the nail plate. Shell nail syndrome is commonly bilateral due to its concurrence with pulmonary, cardiac, and gastrointestinal diseases. [5] The syndrome can be slow to develop, occurring without the patient's notice for multiple months. [4]
There is currently no specific treatment for shell nail syndrome. Within the account of Cornelius and Shelly, the only treatment prescribed, a two-month course of griseofulvin, "failed to produce any change in her nail dystrophy." [2]
Whereas the earliest description of nail clubbing as a sign of disease is from Hippocrates, [4] the diagnostic methods needed to confirm shell nail syndrome (x-ray or removal of the nail plate) [2] have only been used since first described in Cornelius and Shelly's 1967 account: "On review of the world's literature, we failed to find a report of nail dystrophy similar to this one." [2] This first account of the syndrome describes a 37-year-old white female undergoing therapy for bronchiectasis. The doctors noted that "All of the fingernails showed a peculiar deformity characterized by excessive longitudinal curvature of the nail plate." [2] It was noted that the disease was only observed on her fingers and larger toenails, the smaller ones "essentially normal." [2]
Dr. G. F. Donald of St. Peters, South Australia recounts three cases to Cornelius in a 1969 publication: "One was a sporadic case and the others were in twin sisters, both of whom had had lifelong bronchiectasis which we presumed to be due to congenital faults in the bronchial tree. In all three, there had been this progressive dystrophy of the nails which you illustrate so beautifully. We have presumed that the nail dystrophy and the congenital change leading to chronic bronchiectasis are allied, but a lack of clear-cut proof had never bothered to record it." [6] Cornelius provides this as support for the "contention that the nail changes and the lung pathology are related in some unknown way." [6]
A nail is a claw-like plate at the tip of the fingers and toes in most primates. Nails correspond to claws found in other animals. Fingernails and toenails are made of a tough protective protein called alpha-keratin, which is a polymer. Alpha-keratin is found in the hooves, claws and horns of vertebrates.
A nail disease or onychosis is a disease or deformity of the nail. Although the nail is a structure produced by the skin and is a skin appendage, nail diseases have a distinct classification as they have their own signs and symptoms which may relate to other medical conditions. Some nail conditions that show signs of infection or inflammation may require medical assistance.
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. When it occurs together with joint effusions, joint pains, and abnormal skin and bone growth it is known as hypertrophic osteoarthropathy.
Lichen planus (LP) is a chronic inflammatory and immune-mediated disease that affects the skin, nails, hair, and mucous membranes. It is not an actual lichen, and is only named that because it looks like one. It is characterized by polygonal, flat-topped, violaceous papules and plaques with overlying, reticulated, fine white scale, commonly affecting dorsal hands, flexural wrists and forearms, trunk, anterior lower legs and oral mucosa. The hue may be gray-brown in people with darker skin. Although there is a broad clinical range of LP manifestations, the skin and oral cavity remain as the major sites of involvement. The cause is unknown, but it is thought to be the result of an autoimmune process with an unknown initial trigger. There is no cure, but many different medications and procedures have been used in efforts to control the symptoms.
An ingrown nail, also known as onychocryptosis from Greek: ὄνυξ 'nail' and κρυπτός 'hidden', is a common form of nail disease. It is an often painful condition in which the nail grows so that it cuts into one or both sides of the paronychium or nail bed. While ingrown nails can occur in the nails of both the hands and the feet, they occur most commonly with the toenails, and for the most part are only problematic and painful on the big toe.
Beau's lines are deep grooved lines that run from side to side on the fingernail or the toenail. They may look like indentations or ridges in the nail plate. This condition of the nail was named by a French physician, Joseph Honoré Simon Beau (1806–1865), who first described it in 1846.
Paronychia is an inflammation of the skin around the nail, which can occur suddenly, when it is usually due to the bacterium Staphylococcus aureus, or gradually when it is commonly caused by the fungus Candida albicans. The term is from Greek: παρωνυχία from para 'around', onyx 'nail', and the abstract noun suffix -ia.
Onychomycosis, also known as tinea unguium, is a fungal infection of the nail. Symptoms may include white or yellow nail discoloration, thickening of the nail, and separation of the nail from the nail bed. Toenails or fingernails may be affected, but it is more common for toenails. Complications may include cellulitis of the lower leg. A number of different types of fungus can cause onychomycosis, including dermatophytes and Fusarium. Risk factors include athlete's foot, other nail diseases, exposure to someone with the condition, peripheral vascular disease, and poor immune function. The diagnosis is generally suspected based on the appearance and confirmed by laboratory testing.
Onycholysis is a common medical condition characterized by the painless detachment of the nail from the nail bed, usually starting at the tip and/or sides. On the hands, it occurs particularly on the ring finger but can occur on any of the fingernails. It may also happen to toenails.
A subungual hematoma is a collection of blood (hematoma) underneath a toenail or fingernail. It can be extremely painful for an injury of its size, although otherwise it is not a serious medical condition.
A systemic disease is one that affects a number of organs and tissues, or affects the body as a whole.
Nail–patella syndrome is a genetic disorder that results in small, poorly developed nails and kneecaps, but can also affect many other areas of the body, such as the elbows, chest, and hips. The name "nail–patella" can be very misleading because the syndrome often affects many other areas of the body, including even the production of certain proteins. Those affected by NPS may have one or more affected areas of the body, and its severity varies depending on the individual. It is also referred to as iliac horn syndrome, hereditary onychoosteodysplasia, Fong disease or Turner–Kieser syndrome.
Pachyonychia congenita is a rare group of autosomal dominant skin disorders that are caused by a mutation in one of five different keratin genes. Pachyonychia congenita is often associated with thickened toenails, plantar keratoderma, and plantar pain.
Terry's nails is a physical condition in which a person's fingernails or toenails appear white with a characteristic "ground glass" appearance without any lunula. The condition is thought to be due to a decrease in vascularity and an increase in connective tissue within the nail bed. It frequently occurs in the setting of liver failure, cirrhosis, diabetes mellitus, congestive heart failure, hyperthyroidism, or malnutrition. Eighty percent of patients with severe liver disease have Terry's nails, but they are also found in people with kidney failure, in patients with congestive heart failure and are described as a brown arc near the ends of the nails. The recognition of characteristic nail patterns, such as Terry's nails, may be a helpful herald for early diagnosis of systemic diseases. This finding was named for Richard Terry.
Muehrcke's nails or Muehrcke's lines are changes in the fingernail that may be a sign of an underlying medical condition. The term refers to a set of one or more pale transverse bands extending all the way across the nail, parallel to the lunula. In contrast to Beau's lines, they are not grooved, and in contrast to Mees' lines, the thumb is usually not involved.
Periungual warts are warts that cluster around the fingernail or toenail. They appear as thickened, fissured cauliflower-like skin around the nail plate. Periungual warts often cause loss of the cuticle and paronychia. Nail biting increases susceptibility to these warts.
Bart syndrome, also known as aplasia cutis congenita type VI, is a rare genetic disorder characterized by the association of congenital localized absence of skin, mucocutaneous blistering and absent and dystrophic nails.
Koenen's tumor (KT), also commonly termed periungual angiofibroma, is a subtype of the angiofibromas. Angiofibromas are benign papule, nodule, and/or tumor lesions that are separated into various subtypes based primarily on the characteristic locations of their lesions. KTs are angiofibromas that develop in and under the toenails and/or fingernails. KTs were once considered as the same as another subtype of the angiofibromas viz., acral angiofibromas. While the literature may still sometimes regard KTs as acral angiofibromas, acral angiofibromas are characteristically located in areas close to but not in the toenails and fingernails as well as in the soles of the feet and palms of the hands. KTs are here regarded as distinct from acral angiofibromas.
Cooks syndrome is a hereditary disorder which is characterized in the hands by bilateral nail hypoplasia on the thumb, index finger, and middle finger, absence of fingernails (anonychia) on the ring finger and little finger, lengthening of the thumbs, and bulbousness of the fingers. In the feet, it is characterized by absence of toenails and absence/hypoplasia of the distal phalanges. In the second study of this disorder, it was found that the intermediate phalanges, proximal phalanges, and metacarpals were unaffected.
Haim–Munk syndrome is a skin disease caused, like Papillon-Lefevre Syndrome, by a mutation in the cathepsin C gene. One of its features is thick curved finger and toenails.