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Calcinosis may result from a variety of causes such as:
- Trauma to the region
- Inflammation (bug bites, acne)
- Varicose veins
- Infections
- Tumors (malignant or benign)
- Diseases of connective tissue
- Hypercalcemia
- Hyperphosphatemia
Calicinosis cutis is associated with systemic sclerosis.
Calcinosis cutis may be divided into the following types:
- Dystrophic calcinosis cutis
- Metastatic calcinosis cutis
- Iatrogenic calcinosis cutis
- Traumatic calcinosis cutis
- Idiopathic calcinosis cutis
- Idiopathic scrotal calcinosis
- Subepidermal calcified nodule
- Tumoral calcinosis
- Osteoma cutis
A number of conditions may cause the appearance of livedo reticularis:
- Cutis marmorata telangiectatica congenita, a rare congenital condition
- Sneddon syndrome – association of livedoid vasculitis and systemic vascular disorders, such as strokes, due to underlying genetic cause
- Idiopathic livedo reticularis – the most common form of livedo reticularis, completely benign condition of unknown cause affecting mostly young women during the winter: It is a lacy purple appearance of skin in extremities due to sluggish venous blood flow. It may be mild, but ulceration may occur later in the summer.
- Secondary livedo reticularis:
- Vasculitis autoimmune conditions:
- Livedoid vasculitis – with painful ulceration occurring in the lower legs
- Polyarteritis nodosa
- Systemic lupus erythematosus
- Dermatomyositis
- Rheumatoid arthritis
- Lymphoma
- Pancreatitis
- Chronic pancreatitis
- Tuberculosis
- Drug-related:
- Adderall (side effect)
- Amantadine (side effect)
- Bromocriptine (side effect)
- Beta IFN treatment, "i.e." in multiple sclerosis
- Livedo reticularis associated with rasagiline
- Methylphenidate and dextroamphetamine-induced peripheral vasculopathy
- Gefitinib
- Obstruction of capillaries:
- Cryoglobulinaemia – proteins in the blood that clump together in cold conditions
- Antiphospholipid syndrome due to small blood clots
- Hypercalcaemia (raised blood calcium levels which may be deposited in the capillaries)
- Haematological disorders of polycythaemia rubra vera or thrombocytosis (excessive red cells or platelets)
- Infections (syphilis, tuberculosis, Lyme disease)
- Associated with acute renal failure due to cholesterol emboli status after cardiac catheterization
- Arteriosclerosis (cholesterol emboli) and homocystinuria (due to Chromosome 21 autosomal recessive Cystathionine beta synthase deficiency)
- Intra-arterial injection (especially in drug addicts)
- Ehlers-Danlos syndrome – connective tissue disorder, often with many secondary conditions, may be present in all types
- Pheochromocytoma
- Livedoid vasculopathy and its association with factor V Leiden mutation
- FILS syndrome (polymerase ε1 mutation in a human syndrome with facial dysmorphism, immunodeficiency, livedo, and short stature)
- Primary hyperoxaluria, oxalosis (oxalate vasculopathy)
- Cytomegalovirus infection (very rare clinical form, presenting with persistent fever and livedo reticularis on the extremities and cutaneous necrotizing vasculitis of the toes)
- Generalized livedo reticularis induced by silicone implants for soft tissue augmentation
- As a rare skin finding in children with Down syndrome
- Idiopathic livedo reticularis with polyclonal IgM hypergammopathy
- CO angiography (rare, reported case)
- A less common skin lesion of Churg-Strauss syndrome
- Erythema nodosum-like cutaneous lesions of sarcoidosis showing livedoid changes in a patient with sarcoidosis and Sjögren's syndrome
- Livedo vasculopathy associated with IgM antiphosphatidylserine-prothrombin complex antibody
- Livedo vasculopathy associated with plasminogen activator inhibitor-1 promoter homozygosity and prothrombin G20210A heterozygosity
- As a first sign of metastatic breast carcinoma (very rare)
- Livedo reticularis associated with renal cell carcinoma (rare)
- Buerger's disease (as an initial symptom)
- As a rare manifestation of Graves hyperthyroidism
- Associated with pernicious anaemia
- Moyamoya disease (a rare, chronic cerebrovascular occlusive disease of unknown cause, characterized by progressive stenosis of the arteries of the circle of Willis leading to an abnormal capillary network and resultant ischemic strokes or cerebral hemorrhages)
- Associated with the use of a midline catheter
- Familial primary cryofibrinogenemia.
The cause is not well defined. Originally considered idiopathic condition. Now accepted that majority of cases develop from dystrophic calcification of cyst contents.
Normophosphatemic familial tumoral calcinosis is a cutaneous disorder characterized by cutaneous calcification or ossification.
Idiopathic scrotal calcinosis (also known as idiopathic calcified nodules of the scrotum) is a cutaneous condition characterized by calcification of the skin resulting from the deposition of calcium and phosphorus occurring on the scrotum. However, the levels of calcium and phosphate in the blood are normal. Idiopathic scrotal calcinosis typically affects young males, with an onset between adolescence and early adulthood. The scrotal calcinosis appears, without any symptoms, as yellowish nodules that range in size from 1 mm to several centimeters.
Other than identifying and treating any underlying conditions in secondary livedo, idiopathic livedo reticularis may improve with warming the area.
Usually observed at birth or shortly thereafter in 94% of patients, in other reports, patients did not develop skin lesions until 3 months or even 2 years after birth. Females are typically affected more often than males (64%).
The prognosis is favorable in most patients with an isolated cutaneous abnormality. In the majority of cases, both the vivid red marking and the difference in circumference of the extremities regress spontaneously during the first year of life. It is theorized that this may be due to the normal maturation process, with thickening of the epidermis and dermis. Improvements for some patients can continue for up to 10 years, while in other cases, the marbled skin may persist for the patient's lifetime.
One study reported an improvement in lesions in 46% of patients within 3 years. If CMTC persists into adulthood, it can result in complaints due to paresthesia, increased sensitivity to cold and pain, and the formation of ulcers.
Few reports included long-term follow up of CMTC into adolescence and adulthood. While about 50% of patients seem to show definite improvement in the reticular vascular pattern, the exact incidence and cause of persistent cases are unknown.
Osteoma cutis is a cutaneous condition characterized by the presence of bone within the skin in the absence of a preexisting or associated lesion.
The cause of the rare condition of tumoral calcinosis is not entirely understood. It is generally characterized by large, globular calcifications near joints.
Milia-like calcinosis is a cutaneous condition characterized by small, milia-like lesions that develop on the dorsal surface of the hands and the face.
Metastatic calcification involves a systemic calcium excess imbalance, which can be caused by hypercalcemia, kidney failure, milk-alkali syndrome, lack or excess of other minerals, or other causes.
Acquired telangiectasia, not related to other venous abnormalities, for example on the face and trunk, can be caused by factors such as
- Acne rosacea
- Blepharitis
- Environmental damage such as that caused by sun or cold exposure
- Age
- Trauma to skin such as contusions or surgical incisions.
- Radiation exposure such as that experienced during radiotherapy for the treatment of cancer
- Chemotherapy
- Carcinoid syndrome
- Limited systemic sclerosis/scleroderma (a Scleroderma sub-type)
- Chronic treatment with topical corticosteroids may lead to telangiectasia.
- Spider angiomas are a radial array of tiny arterioles that commonly occur in pregnant women and in patients with hepatic cirrhosis and are associated with palmar erythema. In men, they are related to high estrogen levels secondary to liver disease.
- Tempi syndrome
- Smoking
At this time, causes are unknown, but it's said it is not congenital.
CREST syndrome can be noted in up to 10% of patients with primary biliary cirrhosis.
In the past, people used to think that leg varicose veins or telangectasia were caused by high venous pressure or "venous hypertension". However it is now understood that venous reflux disease is usually the cause of these problems (see above for reference for "venous reflux".
Telangiectasia in the legs is often related to the presence of venous reflux within underlying varicose veins. Flow abnormalities within the medium-sized veins of the leg (reticular veins) can also lead to the development of telangiectasia.
Factors that predispose to the development of varicose and telangiectatic leg veins include
- Age: The development of spider veins may occur at any age but usually occurs between 18 and 35 years, and peaks between 50 and 60 years.
- Gender: It used to be thought that females were affected far more than males. However research has shown 79% of adult males and 88% of adult females have leg telangectasia (spider veins).
- Pregnancy: Pregnancy is a key factor contributing to the formation of varicose and spider veins. The most important factor is circulating hormones that weaken vein walls. There's also a significant increase in the blood volume during pregnancy, which tends to distend veins, causing valve dysfunction which leads to blood pooling in the veins. Moreover, later in pregnancy, the enlarged uterus can compress veins, causing higher vein pressure leading to dilated veins. Varicose veins that form during pregnancy may spontaneously improve or even disappear a few months after delivery.
- Lifestyle/occupation: Those who are involved with "prolonged sitting or standing" in their daily activities have an increased risk of developing varicose veins. The weight of the blood continuously pressing against the closed valves causes them to fail, leading to vein distention.
Many conditions affect the human integumentary system—the organ system covering the entire surface of the body and composed of skin, hair, nails, and related muscle and glands. The major function of this system is as a barrier against the external environment. The skin weighs an average of four kilograms, covers an area of two square meters, and is made of three distinct layers: the epidermis, dermis, and subcutaneous tissue. The two main types of human skin are: glabrous skin, the hairless skin on the palms and soles (also referred to as the "palmoplantar" surfaces), and hair-bearing skin. Within the latter type, the hairs occur in structures called pilosebaceous units, each with hair follicle, sebaceous gland, and associated arrector pili muscle. In the embryo, the epidermis, hair, and glands form from the ectoderm, which is chemically influenced by the underlying mesoderm that forms the dermis and subcutaneous tissues.
The epidermis is the most superficial layer of skin, a squamous epithelium with several strata: the stratum corneum, stratum lucidum, stratum granulosum, stratum spinosum, and stratum basale. Nourishment is provided to these layers by diffusion from the dermis, since the epidermis is without direct blood supply. The epidermis contains four cell types: keratinocytes, melanocytes, Langerhans cells, and Merkel cells. Of these, keratinocytes are the major component, constituting roughly 95 percent of the epidermis. This stratified squamous epithelium is maintained by cell division within the stratum basale, in which differentiating cells slowly displace outwards through the stratum spinosum to the stratum corneum, where cells are continually shed from the surface. In normal skin, the rate of production equals the rate of loss; about two weeks are needed for a cell to migrate from the basal cell layer to the top of the granular cell layer, and an additional two weeks to cross the stratum corneum.
The dermis is the layer of skin between the epidermis and subcutaneous tissue, and comprises two sections, the papillary dermis and the reticular dermis. The superficial papillary dermis with the overlying rete ridges of the epidermis, between which the two layers interact through the basement membrane zone. Structural components of the dermis are collagen, elastic fibers, and ground substance. Within these components are the pilosebaceous units, arrector pili muscles, and the eccrine and apocrine glands. The dermis contains two vascular networks that run parallel to the skin surface—one superficial and one deep plexus—which are connected by vertical communicating vessels. The function of blood vessels within the dermis is fourfold: to supply nutrition, to regulate temperature, to modulate inflammation, and to participate in wound healing.
The subcutaneous tissue is a layer of fat between the dermis and underlying fascia. This tissue may be further divided into two components, the actual fatty layer, or panniculus adiposus, and a deeper vestigial layer of muscle, the panniculus carnosus. The main cellular component of this tissue is the adipocyte, or fat cell. The structure of this tissue is composed of septal (i.e. linear strands) and lobular compartments, which differ in microscopic appearance. Functionally, the subcutaneous fat insulates the body, absorbs trauma, and serves as a reserve energy source.
Conditions of the human integumentary system constitute a broad spectrum of diseases, also known as dermatoses, as well as many nonpathologic states (like, in certain circumstances, melanonychia and racquet nails). While only a small number of skin diseases account for most visits to the physician, thousands of skin conditions have been described. Classification of these conditions often presents many nosological challenges, since underlying etiologies and pathogenetics are often not known. Therefore, most current textbooks present a classification based on location (for example, conditions of the mucous membrane), morphology (chronic blistering conditions), etiology (skin conditions resulting from physical factors), and so on. Clinically, the diagnosis of any particular skin condition is made by gathering pertinent information regarding the presenting skin lesion(s), including the location (such as arms, head, legs), symptoms (pruritus, pain), duration (acute or chronic), arrangement (solitary, generalized, annular, linear), morphology (macules, papules, vesicles), and color (red, blue, brown, black, white, yellow). Diagnosis of many conditions often also requires a skin biopsy which yields histologic information that can be correlated with the clinical presentation and any laboratory data.
Phakomatosis pigmentovascularis is a rare neurocutanous condition where there is coexistence of a capillary malformation (port-wine stain) with various melanocytic lesions, including dermal melanocytosis (Mongolian spots), nevus spilus, and nevus of Ota.
Membranous aplasia cutis is a cutaneous condition, a type of aplasia cutis congenita, which can be seen along the embryonic fusion lines of the face.
Beare–Stevenson cutis gyrata syndrome is so rare that a reliable incidence cannot be established as of yet; fewer than 20 patients with the condition have been reported.
The proximal causes of fiddler’s neck are friction and pressure, but both repetitive shearing stress and occlusion with consequent trapping of sweat give rise to progressive damage. This damage along with poor hygiene predisposes the area to local infection, and such infection can progress to scarring and other long-term effects. Hot weather is reported to exacerbate fiddler’s neck, as are tiredness, playing emotional music, and playing in smaller groups where individual stress is higher. Type I hypersensitivity reactions may also be involved, particularly to rosewood and ebony in the chinrest and tailpiece, as well as to varnish of the instrument body when chinrests are not used and to rosin deposits on the instrument and on chin cloths. Nickel or other metal allergies are common causes if the chin rest has a metal clip that comes into constant contact with the skin. Rosin exposure in particular may lead to abietic acid dermatitis.
Syringomas can be found in association with other symptoms as part of a syndrome. Hailey-Hailey disease also known as familial benign chronic pemphigus is a blistering disease that can also include syringomas.
Several systemic syndromes have also been associated with syringoma including diabetes mellitus, down syndrome, Brooke-Spiegler, and Nicolau-Balus. Specifically, diabetes mellitus is strongly associated with clear cell syringoma consisting of nests of clear cells containing glycogen. It is thought that phosphorylase deficiency, resulting from elevated glucose levels seen in diabetes leads to an accumulation of glycogen in the skin and within the clear cells. The incidence of syringomas has been reported in up to 40 percent of people with Down syndrome and can be associated with a condition calcinosis cutis which requires prompt medical attention. Brooke-Spiegler syndrome is a rare autosomal dominant syndrome with cutaneous manifestations including syringomas and trichoepitheliomas. Nicolau-Balus is a rare autosomal dominant disorder consisting of atrophoderma vermiculata and syringomas.
Fiddler’s neck does not usually form unless the musician is practicing or playing for more than a few hours each day, and only seems to develop after a few years of serious playing. Thus, when not infected or otherwise problematic, fiddler’s neck may be known as a benign practice mark and may be worn proudly as an indication of long hours of practice. Blum & Ritter (1990) found that 62% of 523 professional violinists and violists in West Germany experienced fiddler’s neck, with the percentage among violists being higher (67%) than among violinists (59%). Viola players are believed to be more predisposed to developing fiddler’s neck than violinists because the viola is larger and heavier, but this has not been empirically confirmed.
The development of fiddler’s neck does not depend on preexisting skin problems, and Blum & Ritter find that only 23% of men and 14% of women in their study reported cutaneous disorders in other parts of the face (mainly acne and eczema) that were independent of playing the violin or viola. Fiddler’s neck may exacerbate existing acne, but acne may also be limited solely to the lesion and not appear elsewhere. Nonetheless, musicians with underlying dermatologic diseases like acne and eczema are more endangered by fiddler’s neck than others. Males may develop folliculitis or boils due to involvement of beard hair.
Papuloerythroderma of Ofuji is a rare disorder most commonly found in Japan, characterized by pruritic papules that spare the skinfolds, producing bands of uninvolved cutis, creating the so-called deck-chair sign. Frequently there is associated blood eosinophilia. Skin biopsies reveal a dense lymphohistiocytic infiltrate, eosinophils in the papillary dermis, and increased Langerhans cells (S-100 positive). Systemic steroids are the treatment of choice and may result in long-term remissions.
It was characterized in 1984.
Use of PUVA in treatment has been described.