| Paraneoplastic acrokeratosis | |
|---|---|
| Other names | Acrokeratosis paraneoplastica of Bazex and Acrokeratosis neoplastica) [1] |
| Specialty | Dermatology  |
Paraneoplastic acrokeratosis, or Bazex syndrome is a cutaneous condition characterized by psoriasiform changes of hands, feet, ears, and nose, with involvement of the nails and periungual tissues being characteristic and indistinguishable from psoriatic nails. The condition is associated with carcinomas of the upper aerodigestive tract. [2] : 665
A case was first reported by Henri Gougerot and C. Grupper in 1922. [3] [4] Andre Bazex described it as a paraneoplastic syndrome in 1965. [3] [5]
Lichen planus (LP) is a chronic inflammatory and autoimmune disease that affects the skin, nails, hair, and mucous membranes. It is not an actual lichen, but is named for its appearance. 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.
Palmoplantar keratodermas are a heterogeneous group of disorders characterized by abnormal thickening of the stratum corneum of the palms and soles.
Ichthyosis acquisita is a skin condition clinically and histologically similar to ichthyosis vulgaris.
Keratoderma is a hornlike skin condition.
Muir–Torre syndrome is a rare hereditary, autosomal dominant cancer syndrome that is thought to be a subtype of HNPCC. Individuals are prone to develop cancers of the colon, genitourinary tract, and skin lesions, such as keratoacanthomas and sebaceous tumors. The genes affected are MLH1, MSH2, and more recently, MSH6, and are involved in DNA mismatch repair.
A paraneoplastic syndrome is a syndrome that is the consequence of a tumor in the body. It is specifically due to the production of chemical signaling molecules by tumor cells or by an immune response against the tumor. Unlike a mass effect, it is not due to the local presence of cancer cells.
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.
Florid cutaneous papillomatosis (FCP), is an obligate paraneoplastic syndrome.
Splinter hemorrhages are tiny blood clots that tend to run vertically under the nails. Splinter hemorrhages are not specific to any particular condition, and can be associated with subacute infective endocarditis, scleroderma, trichinosis, systemic lupus erythematosus (SLE), rheumatoid arthritis, psoriatic nails, antiphospholipid syndrome, haematological malignancy, and trauma. At first they are usually plum-colored, but then darken to brown or black in a couple of days. In certain conditions, clots can migrate from the affected heart valve and find their way into various parts of the body. If this happens in the finger, it can cause damage to the capillaries resulting in a splinter hemorrhage.
In racquet nails, the nail plate is flattened, the end of the thumb is widened and flattened, and the distal phalanx is abnormally short. In racquet nails, the width of the nail bed and nail plate is greater than their length. The condition is painless and asymptomatic.
Schöpf–Schulz–Passarge syndrome is an autosomal recessive condition with punctate symmetric palmoplantar keratoderma, with the keratoderma and fragility of the nails beginning around age 12. In addition to palmoplantar keratoderma, other symptoms include hypodontia, hypotrichosis, nail dystrophies, and eyelid cysts. Patients may also develop syringofibroadenoma and squamous cell carcinomas.
Gougerot–Blum syndrome is a variant of pigmented purpuric dermatitis, a skin condition characterized by minute, rust-colored to violaceous, lichenoid papules that tend to fuse into plaques of various hues. Relative to other variants, it is characterized clinically by a male predominance, pruritus, with a predilection for the legs, and histologically, it features a densely cellular lichenoid infiltrate.
Tripe palms, also known as acanthosis palmaris, is a medical sign characterized by thick ridged velvety palms, typically as part of a paraneoplastic syndrome. It resembles the lining of the stomach of some animals (tripe). Other signs that may be noted at the same time include most frequently acanthosis nigricans (AN), and less commonly finger clubbing and Leser-Trélat sign.
Paraneoplastic keratoderma is a cutaneous condition characterized by a hornlike skin texture associated with an internal malignancy.
Helene Ollendorff Curth was a German-American dermatologist, known for her studies on acanthosis nigricans (AN) and introducing Curth's criteria, a set of characteristics for associating skin signs as markers for internal cancers. She is named in two rare inherited skin diseases, the Buschke–Ollendorff syndrome and Ichthyosis Hystrix, Curth-Macklin Type. A medical sign in secondary syphilis, known as the Ollendorff sign, and one form of measuring clubbed fingers, known as the Curth's angle, are named for her.