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Frontal Fibrosing Alopecia has been most often reported in post-menopausal women with higher levels of affluence and a negative smoking history. Autoimmune disease is found in 30% of patients.
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.
Fibromyalgia was found in 9% of adult patients relative to 0.03% in the general population with a link common to IBD. Concurrent IBS is found in 30% to 70%. Small intestinal bacterial overgrowth is associated is common with a transient response to antimicrobial therapy.
Adenocarcinoma of the bowel has been associated with coeliac disease.
Anetoderma (also known as "Anetoderma maculosa," "Anetoderma maculosa cutis," "Atrophia maculosa cutis," and "Macular atrophy") is a localized laxity of the skin with herniation or outpouching resulting from abnormal dermal elastic tissue. Anetoderma comes in three types:
- "Primary anetoderma"
- Jadassohn–Pellizzari anetoderma is a benign condition with focal loss of dermal elastic tissue. Jadassohn-Pellizzari is one of two major classifications of primary anetoderma, the other being Schweninger–Buzzi anetoderma. The difference between the two is that Jadassohn–Pellizzari anetoderma is preceded by inflammatory lesions.
- Schweninger–Buzzi anetoderma is a cutaneous condition characterized by loss of dermal elastic tissue.
- "Secondary anetoderma"
- "Familial anetoderma"
Atrophia Maculosa Varioliformis Cutis (AMVC) is a condition involving spontaneous scarring, specifically depressed scars on the face occurring over a period of months to years. It appears to only affect children and young adults, is considered to be quite rare, normally occurs on the cheeks, temple area and forehead, and is not well understood nor presently treatable. Case reports indicate the scars deepen over time but remain relatively superficial, and with the frequency of new scar appearance diminishing over time.
AMVC is quite difficult to diagnose, for reasons including the depressed box and ice pick scars being very similar to that caused by Acne vulgaris. A confident diagnosis can be made if such scars recently appeared without present acne and without a history of acne. Otherwise the correct diagnosis is usually not made, and even doing so provides little benefit as there is no treatment. It has been suggested in case reports that the condition, although rare, is likely underreported.
Although the pathogenesis of Frontal Fibrosing Alopecia is poorly understood, autoimmune reaction and hormonal factors may play a role.
Getting a regular eye exam may play a role in identifying the signs of some systemic diseases. "The eye is composed of many different types of tissue. This unique feature makes the eye susceptible to a wide variety of diseases as well as provides insights into many body systems. Almost any part of the eye can give important clues to the diagnosis of systemic diseases. Signs of a systemic disease may be evident on the outer surface of the eye (eyelids, conjunctiva and cornea), middle of the eye and at the back of the eye (retina)."
Since 500 B.C., some researchers have believed that the physical condition of the fingernails and toenails can indicate various systemic diseases. Careful examination of the fingernails and toenails may provide clues to underlying systemic diseases , since some diseases have been found to cause disruptions in the nail growth process. The nail plate is the hard keratin cover of the nail. The nail plate is generated by the nail matrix located just under the cuticle. As the nail grows, the area closest to becoming exposed to the outside world (distal) produces the deeper layers of the nail plate, while the part of the nail matrix deeper inside the finger (proximal) makes the superficial layers. Any disruption in this growth process can lead to an alteration in the shape and texture.
For example, pitting looks like depressions in the hard part of the nail. Pitting is to be associated with psoriasis, affecting 10% - 50% of patients with that disorder. Pitting also may be caused by a variety of systemic diseases, including reactive arthritis and other connective tissue disorders, sarcoidosis, pemphigus, alopecia areata, and incontinentia pigmenti. Because pitting is caused by defective layering of the superficial nail plate by the proximal nail matrix, any localized dermatitis (e.g., atopic dermatitis or chemical dermatitis) that disrupts orderly growth in that area also can cause pitting.
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A systemic disease is one that affects a number of organs and tissues, or affects the body as a whole.
Infection-related cutaneous conditions may be caused by bacteria, fungi, yeast, viruses, or parasites.
There are various causes of madarosis.
- Ophthalmological conditions: blepharitis is an infection of the eyelid. Anterior blepharitis is either "staphylococcal blepharitis,"or "seborrhoeic blepharitis" and posterior blepharitis is due to the meibomian gland.
- Dermatologic conditions: there are multiple types of dermatological conditions that can result in madarosis. These include Atopic dermatitis, Seborrhoeic dermatitis atopic dermatitis, and Psoriasis on the eyelids can result in madarosis. Others include: frontal fibrosing alopecia, ulerythema ophryogenes, acne rosacea, telogen effluvium, follicular mucinosis, and cutaneous sarcoidosis.
- Nutritional defects: Severe malnutrition can cause chronic hair loss. Hypoproteinemia causes hair loss by early onset of telogen. Zinc deficiencies like acrodermatitis enteropathica, can lead to the loss of eyebrow/eyelash hair. Other deficiencies like biotin and iron make it possible for loss of hair as well.
- Infections: There are many bodily infections that can cause the loss of eyelashes/eyebrows. The most common infection may be leprosy, such as lepromatous leprosy. Syphilis or other viral infections like herpes or HIV can cause the loss of eye hair as well. Fungal infections, like paracoccidioidomycosis, trichophyton, or microsporum, are also possible infection causes.
- Trauma: Most trauma injuries cause madarosis from the psychological standpoint, known as trichotillomania
- Drugs/Medications: Crack cocaine or chemotherapy drugs. Other drugs include:propranolol, valproic acid, barbiturates, MMR vaccine, botulinum toxin, epinephrine, antithyroid drugs, anticoagulants, and lipid-lowering drugs
- Genetics
- Autoimmune disorders: alopecia areata, discoid lupus erythematosus, chronic cutaneous lupus erythmatosus, Graham-Little syndrome, and Parry Romberg syndrome
- Other diseases: hypothyroidism, hyperthyroidism, hypoparathyroidism, hypopituitarism, and amyloidosis
There only prevention method is determining the underlying condition before treatment options are too late.
Geographic tongue is a common condition, affecting 2-3% of the adult general population, although other sources report a prevalence of up to 14%. It is one of the most common tongue disorders that occurs in children. The condition often starts in childhood, sometimes at an early age, but others report that the highest incidence occurs in the over 40 age group. Females are sometimes reported to be more commonly affected than males, in a 2:1 ratio, although others report that the gender distribution is equal.
Ophiasis is a form of alopecia areata characterized by the loss of hair in the shape of a wave at the circumference of the head.
It gets its name from "ophis", which is the Greek word for snake, because of the apparent similarity to a snake-shape and the pattern of hair loss.
The term "sisaipho" is used to characterize the inverse pattern. Sisaipho is the reverse spelling of ophiasis. It is also called "ophiasis inversus".
The cause is unknown. Geographic tongue does not usually cause any symptoms, and in those cases where there are symptoms, an oral parafunctional habit may be a contributory factor. Persons with parafunctional habits related to the tongue may show scalloping on the sides of the tongue (crenated tongue). Some suggest that hormonal factors may be involved, because one reported case in a female appeared to vary in severity in correlation with oral contraceptive use. People with geographic tongue frequently claim that their condition worsens during periods of psychologic stress. Geographic tongue is inversely associated with smoking and tobacco use. Sometimes geographic tongue is said to run in families, and it is reported to be associated with several different genes, though studies show family association may also be caused by similar diets. Some have reported links with various human leukocyte antigens, such as increased incidence of HLA-DR5, HLA-DRW6 and HLA-Cw6 and decreased incidence in HLA-B51. Vitamin B2 deficiency (ariboflavinosis) can cause several signs in the mouth, possibly including geographic tongue, although other sources state that geographic tongue is not related to nutritional deficiency. Fissured tongue often occurs simultaneously with geographic tongue, and some consider fissured tongue to be an end stage of geographic tongue.
In the past, some research suggested that geographic tongue was associated with diabetes, seborrheic dermatitis and atopy, however newer research does not corroborate these findings. Others suggest allergy as a major factor, e.g. to nickel sulphate. Some studies have reported a link between geographic tongue and psoriasis, although 90% of children who are diagnosed with geographic tongue do not develop psoriasis. Again however, modern research studies do not support any link between psoriasis and geographic tongue. Lesions that are histologically indistinguishable from geographic tongue may also be diagnosed in reactive arthritis (arthritis, uveitis/conjunctivitis and urethritis).
Trachyonychia, sometimes called sandpapered nails, is a condition characterized by rough accentuated linear ridges (longitudinal striations) on the nails of the fingers and toes. When the condition occurs on all the twenty nails of the fingers and toes, it is known as twenty-nail dystrophy, most evident in childhood, favoring males.
Trachyonychia causes the nails to become opalescent, thin, dull, fragile, and finely longitudinally ridged, and, as a result, distally notched. It can be a manifestation of lichen planus, psoriasis, alopecia areata, immunoglobulin A deficiency, atopic dermatitis, and ichthyosis vulgaris.
"The longitudinal striations can occur as a normal part of the aging process", and not until the nails start to thin and get a sandpaper look is the condition called trachonychia. The nails are opalescent and frequently are brittle and split at the free margin. There has been evidence of the condition as a cutaneous manifestation of lichen planus. It has also been associated with other diseases such as eczema, psoriasis, alopecia areata, and atopic dermatitis. Trachonychia is often seen in vitiligo patients – suggesting that they are more susceptible to this condition.
Causes of noncicatricial alopecia include:
- Alopecia areata
- Anagen effluvium
- Androgenetic alopecia
- Dermatopathia pigmentosa reticularis
- Telogen effluvium
- Trichotillomania (Trichotillosis)
There are two major pathways. In the non-scarring pathway, the hair follicles remain intact which could potentially be reversed with the proper diagnosis and treatments. In the Scaring pathway, the follicles are permanently lost due to tissue damage, autrophy, or inflammation.
The severity of the pre-existing condition determines which type of madarosis occurs.
- Ophthalmologic conditions: there are multiple types of infections and are common. The gray line of the eye is a line that divides the eyelid into parts: anterior part is skin and muscle while posterior is tarsus and conjunctiva. The blepharitis is classified based on the type of eyelid involvement. Anterior blepharitis is either "staphylococcal blepharitis,"or "seborrhoeic blepharitis" which have symptoms of the presence of scales that are along the hair shaft. Posterior blepharitis is usually due to meibomian gland dysfunction.
- Dermatologic conditions: there are multiple types of dermatological conditions that can result in madarosis dependent on the location. Atopic dermatitis is associated with allergic disorders and affects the lower eyelid. Seborrhoeic dermatitis can be seen as the scaling of the eyebrows. Loss of eyebrows is commonly seen from both atopic and seborrhoeic dermatitis due to continuous scratching/touching. Psoriasis on the eyelids can result in madarosis. Others include: frontal fibrosing alopecia, ulerythema ophryogenes, acne rosacea, telogen effluvium, follicular mucinosis, and cutaneous sarcoidosis.
- Nutritional Defects: Zinc is important in hair growth and the lack of zinc can cause hair loss, specifically madarosis.The main proteins in hair are biotin and iron so deficiencies would result in a loss of hair.
- Infections: Unilateral madarosis (only one eye) may occur in tuberculoid leprosy from granulomatous infiltration of hair follicles ultimately destroying them. Infections like syphilis by causing a moth-eaten appearance of the eyebrow hair loss. Viral infections like herpes or HIV can cause scarring of the eyelid, causing loss of hair. Various fungal infections like paracoccidioidomycosis can cause lesions and changes in the hair locations of the eyelid.
- Trauma: Trichotillomania is a psychological disorder where the hair is pulled out or breakage occurs when anxious. Both trichoteiromania (hair loss from constant rubbing) and trichotemnomania (hair loss from constant shaving of the hair) can result in hair loss.
- Drugs/Medications: The heart medication amiodarone has been reported to commonly cause loss of eyelashes and eyebrows. Cocaine abuse has shown hair loss due to the hot vapors traveling up causing burning of the hair of the eyebrows or eyelashes. Radiotherapy and chemotherapy can cause hair loss due to the eradication of the hair cells, especially when used to treat ocular tumors.
- Genetics: Some of the diseases listed in the causes can be inherited.
Alopecia universalis can occur at any age, and is currently believed to be an autoimmune disorder, in which a person's immune system attacks the hair follicles. Genetic factors may contribute to AU as approximately 20% of those affected have a family member with alopecia.
Non scarring hair loss, also known as noncicatricial alopecia, is the loss of hair without any scarring being present. This is in contrast to scarring hair loss.
Other causes of hair loss include:
- Alopecia mucinosa
- Biotinidase deficiency
- Chronic inflammation
- Diabetes
- Lupus erythematosus
- Pseudopelade of Brocq
- Telogen effluvium
- Tufted folliculitis
The condition affects 0.1%–0.2% of the population, and occurs equally in both males and females. Alopecia areata occurs in people who are otherwise healthy and have no other skin disorders. Initial presentation most commonly occurs in the late teenage years, early childhood, or young adulthood, but can happen at any ages. Patients also tend to have a slightly higher incidence of conditions related to the immune system, such as asthma, allergies, atopic dermatitis, and hypothyroidism.
Variations in genes that are part of the immune system or part of melanocytes have both been associated with vitiligo. It is also thought to be caused by the immune system attacking and destroying the melanocytes of the skin. A genomewide association study found approximately 36 independent susceptibility loci for generalized vitiligo.
Alopecia universalis (AU) or alopecia areata universalis is a medical condition involving loss of all hair, including eyebrows and eyelashes. It is an advanced form of alopecia areata.
Although multiple hypotheses have been suggested as potential triggers that cause vitiligo, studies strongly imply that changes in the immune system are responsible for the condition. Vitiligo has been proposed to be a multifactorial disease with genetic susceptibility and environmental factors both thought to play a role.
The TYR gene encodes the protein tyrosinase, which is not a component of the immune system, but is an enzyme of the melanocyte that catalyzes melanin biosynthesis, and a major autoantigen in generalized vitiligo. The NIH states that sunburns can cause the disease but there is not good evidence to support this.
Preliminary evidence suggests a possible association with eating gluten.
Research is looking into connections between hair loss and other health issues. While there has been speculation about a connection between early-onset male pattern hair loss and heart disease, a review of articles from 1954 to 1999 found no conclusive connection between baldness and coronary artery disease. The dermatologists who conducted the review suggested further study was needed.
Environmental factors are under review. A 2007 study indicated that smoking may be a factor associated with age-related hair loss among Asian men. The study controlled for age and family history, and found statistically significant positive associations between moderate or severe male pattern hairloss and smoking status.
Vertex baldness is associated with an increased risk of coronary heart disease (CHD) and the relationship depends upon the severity of baldness, while frontal baldness is not. Thus, vertex baldness might be a marker of CHD and is more closely associated with atherosclerosis than frontal baldness.