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AP is characterized by itchy, inflamed papules, nodules, and plaques on the skin. Lesions typically appear hours or days after exposure of the skin to UV light, and follow a general pattern of sun-exposed areas. The face, neck, arms, hands, and legs are often affected, although lesions sometimes appear on skin that is covered by clothing and thus not exposed to UV light, thus making AP somewhat difficult to diagnose.
AP is a chronic disease, and symptoms usually worsen in the spring and summer as the day lengthens and exposure to sunlight increases.
Actinic prurigo (AP) was first described by Escalona in Mexico, in 1954.
Prurigo is an itchy eruption of the skin.
Specific types include:
- Prurigo nodularis
- Actinic prurigo
- Besnier's prurigo (a specific type of atopic dermatitis).
Actinic keratoses ("AKs") most commonly present as a white, scaly plaque of variable thickness with surrounding redness; they are most notable for having a sandpaper-like texture when felt with a gloved hand. Skin nearby the lesion often shows evidence of solar damage characterized by notable pigmentary alterations, being yellow or pale in color with areas of hyperpigmentation; deep wrinkles, coarse texture, purpura and ecchymoses, dry skin, and scattered telangiectasias are also characteristic. Photoaging leads to an accumulation of oncogenic changes, resulting in a proliferation of mutated keratinocytes that can manifest as AKs or other neoplastic growths. With years of sun damage, it is possible to develop multiple AKs in a single area on the skin.
The lesions are usually asymptomatic, but can be tender, itch, bleed, or produce a stinging or burning sensation. AKs are typically graded in accordance with their clinical presentation: Grade I (easily visible, slightly palpable), Grade II (easily visible, palpable), and Grade III (frankly visible and hyperkeratotic).
Prurigo simplex is a chronic, itchy, idiopathic skin condition characterized by extremely itchy skin nodules and lesions. Typically, there is no known direct cause of prurigo simplex, but some factors are known to trigger or aggravate it. This condition falls between chronic and acute, sometimes transitioning into a chronic condition. Many people experience a recurrence of the condition after periods of remission. Middle-aged patients are the most prone age group to this condition.
The most common prurigo simplex symptoms are skin nodules resembling insect bites that are intensely itchy. These nodules are frequently scratched open, becoming lesions that continue to itch. Sometimes the skin thickens and becomes discolored around the nodules. The scalp, arms, legs and trunk of the body are the most frequent sites of the bumps and lesions. Itching can become severe and habitual, worsening the condition and possibly causing infections in the open sores.
Sometimes the nodules become less itchy and eventually disappear leaving a discolored area or scar tissue. The same nodules can persist for months or even years, though, without healing. Patients may experience a remission but then relapse with new nodules forming. The condition might also become chronic, with no periods of improvement and relief.
Treatment is challenging, with narrow band UVB or pimozide sometimes helpful.
Actinic keratoses can have various clinical presentations, often characterized as follows:
- Classic (or common): Classic AKs present as white, scaly macules, papules or plaques of various thickness, often with surrounding erythema. They are usually 2-6mm in diameter but can sometimes reach several centimeters in diameter.
- Hypertrophic (or hyperkeratotic): Hypertrophic AKs (HAKs) appear as a thicker scale or rough papule or plaque, often adherent to an erythematous base. Classic AKs can progress to become HAKs, and HAKs themselves can be difficult to distinguish from malignant lesions.
- Atrophic: Atrophic AKs lack an overlying scale, and therefore appear as a nonpalpable change in color (or macule). They are often smooth and red, and are less than 10mm in diameter.
- AK with cutaneous horn: A cutaneous horn is a keratinic projection with its height at least one-half of its diameter, often conical in shape. They can be seen in the setting of actinic keratosis as a progression of an HAK, but are also present in other skin conditions. 38–40% of cutaneous horns represent AKs.
- Pigmented AK: Pigmented AKs are rare variants that often present as macules or plaques that are tan to brown in color. They can be difficult to distinguish from a solar lentigo or lentigo maligna.
- Actinic cheilitis: When an AK forms on the lip, it is called actinic cheilitis. This usually presents as a rough, scaly patch on the lip, often accompanied by the sensation of dry mouth and symptomatic splitting of the lips.
- Bowenoid AK: Usually presents as a solitary, erythematous, scaly patch or plaque with well-defined borders. Bowenoid AKs are differentiated from Bowen's disease by degree of epithelial involvement as seen on histology.
The presence of ulceration, nodularity, or bleeding should raise concern for malignancy. Specifically, clinical findings suggesting an increased risk of progression to squamous cell carcinoma can be recognized as "IDRBEU": I (induration /inflammation), D (diameter > 1 cm), R (rapid enlargement), B (bleeding), E (erythema) and U (ulceration). AKs are usually diagnosed clinically, but because they are difficult to clinically differentiate from squamous cell carcinoma, any concerning features warrant biopsy for diagnostic confirmation.
AC almost always affects the lower lip and only rarely the upper lip, probably because the lower lip is more exposed to the sun. In the unusual cases reported where it affects the upper lip, this may be due to upper lip prominence. The commissures (corners of the mouth) are not usually involved.
Affected individuals may experience symptoms such as a dry sensation and cracking of the lips.
It is usually painless and persistent.
The appearance is variable. White lesions indicate hyperkeratosis. Red, erosiive or ulcerative lesions indicate atrophy, loss of epithelium and inflammation. Early, acute lesions may be erythematous (red) and edematous (swollen). With months and years of sun exposure, the lesion becomes chronic and may be grey-white in color and appear dry, scaly and wrinkled.
There is thickening whitish discoloration of the lip at the border of the lip and skin. There is also a loss of the usually sharp border between the red of the lip and the normal skin, known as the vermillion border. The lip may become scaly and indurated as AC progresses.
When palpated, the lip may have a texture similar to rubbing the gloved finger along sandpaper.
AC may occur with skin lesions of actinic keratosis or skin cancer elsewhere, particularly on the head and neck since these are the most sun exposed areas. Rarely it may represent a genetic susceptibility to light damage (e.g. xeroderma pigmentosum or actinic prurigo).
Chronic and repetitive scratching, picking, or rubbing of the nodules may result in permanent changes to the skin, including nodular lichenification, hyperkeratosis, hyperpigmentation, and skin thickening. Unhealed, excoriated lesions are often scaly, crusted or scabbed. Many patients report a lack of wound healing even when medications relieve the itching and subsequent scratching.
Patients often:
- seek treatment during middle-age, although PN can occur at any age.
- have a history of chronic severe pruritus.
- have a significant medical history for unrelated conditions.
- suffer from liver or kidney dysfunctions.
- suffer secondary skin infections.
- have a personal or family history of atopic dermatitis.
- have other autoimmune disorders.
- have low vitamin D levels.
The cause of prurigo nodularis is unknown, although other conditions may induce PN. PN has been linked to Becker's nevus, linear IgA disease, an autoimmune condition, liver disease and T cells. Systemic pruritus has been linked to cholestasis, thyroid disease, polycythaemia rubra vera, uraemia, Hodgkins disease, HIV and other immunodeficiency diseases. Internal malignancies, liver failure, renal failure, and psychiatric illnesses have been considered to induce PN, although more recent research has refuted a psychiatric cause for PN. Patients report an ongoing battle to distinguish themselves from those with psychiatric disorders such as delusions of parasitosis and other psychiatric conditions.
Actinic elastosis usually appears as thickened, dry, wrinkled skin. Several clinical variants have been recorded. One of the most readily identifiable is the thickened, deeply fissured skin seen on the back of the chronically sun-exposed neck, known as cutis rhomboidalis nuchae. These features are a part of the constellation of changes that are seen in photoaged skin.
Actinic cheilitis (abbreviated to AC, also termed actinic cheilosis, actinic keratosis of lip, solar cheilosis, sailor's lip, farmer's lip), is cheilitis (lip inflammation) caused by long term sunlight exposure. Essentially it is a burn, and a variant of actinic keratosis which occurs on the lip. It is a premalignant condition, as it can develop into squamous cell carcinoma (a type of mouth cancer).
Photosensitivity with HIV infection is a skin condition resembling polymorphous light eruption, actinic prurigo, or chronic actinic dermatitis, seen in about 5% of HIV-infected people.
Actinic elastosis, also known as solar elastosis, is an accumulation of abnormal elastin (elastic tissue) in the dermis of the skin, or in the conjunctiva of the eye, which occurs as a result of the cumulative effects of prolonged and excessive sun exposure, a process known as photoaging.
Chronic actinic dermatitis (also known as "Actinic reticuloid," "Chronic photosensitivity dermatitis," "Persistent light reactivity," and "Photosensitive eczema") is a condition where a subject's skin becomes inflamed due to a reaction to sunlight or artificial light. Patients often suffer from other related conditions of the skin that cause dermatitis in response to a variety of stimuli (e.g., flowers, sunscreens, cosmetics, etc.).
Diagnosis can occur at any age, ranging from soon after birth to adulthood. A GP may refer a patient to a dermatologist if the condition is not showing clear symptoms, and a variety of tests - usually completed at a hospital - can then determine the exact nature and cause of the patient's condition.
Reactions, which vary depending on the severity of the case, include rashes, flared 'bumpy' patches, affected areas being extremely hot to touch, and outbreaks shortly (or within 24 hours) after direct or indirect exposure to UVA and/or UVB light. The skin most likely reacts on the upper chest, hands and face, however it is not unlikely for reactions to happen all over the body. The patient may feel burning, stinging or throbbing sensations in these areas, which causes mild, yet uncomfortable pain.
Disseminated superficial actinic porokeratosis (DSAP) is a non-contagious skin condition with apparent genetic origin in the SART3 gene. It most often presents in sun-exposed areas of the body. Some DSAP cases have been reported in patients with acute immune compromised situations, particularly in the elderly. For those with sun damaged skin, the lesions usually begin to appear in the patient's 20s and increase in number and visibility in the 40s or 50s. Commonly, though not always, the number and visibility of lesions is in direct proportion to the amount of sun damage to the affected area.
Lesions generally are characterized by an irregularly shaped thread-like ring that is usually the size of a pencil eraser, though lesions vary and may be half or double that size. The thread-like ring is very thin, much like fabric thread for sewing, and raised such that it is both palpable and visible. The interior of the ring may be rough like sandpaper, or smooth. The interior is often discolored, though colors vary from patient to patient. Lesions, due to their vascular nature, can also vary according to body temperature, environmental temperatures, and other external stimuli. The internal ring color is most often reddish, purplish, pink, or brown.
Some patients report itching and irritation associated with the condition, and many report no notable sensation. Although no known hormonal link has been found, DSAP occurs more commonly in women.
A study in 2000 was done on a Chinese family, in which a locus for a gene was located.
Keratosis (from "keratinocyte", the prominent cell type in the epidermis, and , abnormal) is a growth of keratin on the skin or on mucous membranes. More specifically, it can refer to:
- actinic keratosis (also known as solar keratosis)
- hydrocarbon keratosis
- keratosis pilaris (KP, also known as follicular keratosis)
- seborrheic keratosis
Actinic keratoses are pre-malignant growths. Seborrheic keratoses are not pre-malignant.
Prurigo pigmentosa is a rare skin condition of unknown cause, characterized by the sudden onset of erythematous papules that leave a reticulated hyperpigmentation when they heal.
Most cases of leukoplakia cause no symptoms, but infrequently there may be discomfort or pain. The exact appearance of the lesion is variable. Leukoplakia may be white, whitish yellow or grey. The size can range from a small area to much larger lesions. The most common sites affected are the buccal mucosa, the labial mucosa and the alveolar mucosa, although any mucosal surface in the mouth may be involved. The clinical appearance, including the surface texture and color, may be homogenous or non-homogenous (see: classification). Some signs are generally associated with a higher risk of cancerous changes (see: prognosis).
Leukoplakia may rarely be associated with esophageal carcinoma.
In the context of lesions of the mucous membrane lining of the bladder, leukoplakia is a historic term used to describe a visualized white patch which histologically represents keratinization in an area of squamous metaplasia. The symptoms may include frequency, suprapubic pain (pain felt above the pubis), hematuria (blood in the urine), dysuria (difficult urination or pain during urination), urgency, and urge incontinence. The white lesion may be seen during cystoscopy, where it appears as a whitish-gray or yellow lesion, on a background of inflamed urothelium and there may be floating debris in the bladder. Leukoplakia of the bladder may undergo cancerous changes, so biopsy and long term follow up are usually indicated.
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.
Actinic granuloma (also known as "O'Brien granuloma") is a cutaneous condition characterized histologically by a dermal infiltrate of macrophages.
Actinic granuloma is an asymptomatic granulomatous reaction that affects sun-exposed skin, most commonly on the face, neck, and scalp.
It is characterized by annular or polycyclic lesions that slowly expand centrifugally and have an erythematous elevated edge and a hypopigmented, atrophic center.
Advise to reduce exposure to the sun and to use sunscreen.
Treatment with topical halometasone cream, pimecrolimus cream.
Porokeratosis may be divided into the following clinical types:
- "Plaque-type porokeratosis" (also known as "Classic porokeratosis" and "Porokeratosis of Mibelli") is characterized by skin lesions that start as small, brownish papules that slowly enlarge to form irregular, annular, hyperkeratotic or verrucous plaques. Sometimes they may show gross overgrowth and even horn-like structures may develop. Skin malignancy, although rare, is reported from all types of porokeratosis. Squamous cell carcinomata have been reported to develop in Mibelli's type porokeratosis over partianal areas involving anal mucosa. This was the first report mentioning mucosal malignancy in any form of porokeratosis.
- "Disseminated superficial porokeratosis" is a more generalized processes and involves mainly the extremities in a bilateral, symmetric fashion. In about 50% of cases, skin lesions only develop in sun-exposed areas, and this is referred to as "disseminated superficial actinic porokeratosis"
- "Porokeratosis palmaris et plantaris disseminata" is characterized by skin lesions that are superficial, small, relatively uniform, and demarcated by a distinct peripheral ridge of no more than 1mm in height.
- "Linear porokeratosis" is characterized clinically skin lesions are identical to those of classic porokeratosis, including lichenoid papules, annular lesions, hyperkeratotic plaques with central atrophy, and the characteristic peripheral ridge.
- "Punctate porokeratosis" is a skin condition associated with either classic porokeratosis or linear porokeratosis types of porokeratosis, and is characterized by multiple, minute, and discrete punctate, hyperkeratotic, seed-like skin lesions surrounded by a thin, raised margin on the palms and soles.
- "Porokeratosis plantaris discreta" is a skin condition that occurs in adults, with a 4:1 female preponderance, characterized by a sharply marginated, rubbery, wide-based papules. It is also known as "Steinberg's lesion". It was characterized in 1970.
Porokeratosis is a specific disorder of keratinization that is characterized histologically by the presence of a cornoid lamella, a thin column of closely stacked, parakeratotic cells extending through the stratum corneum with a thin or absent granular layer.
Prurigo gestationis (also called "Besnier prurigo", "Early-onset prurigo of pregnancy," "Linear IgM dermatosis of pregnancy," "Papular dermatitis of pregnancy," "Prurigo of pregnancy", and "Spangler's papular dermatitis of pregnancy") is an eruption consisting of pruritic, excoriated papules of the proximal limbs and upper trunk, most often occurring between the 20th and 34th week of gestation.
The exact etiology is unknown, but it is considered likely to be a flareup of atopic dermatitis during pregnancy.
It is sometimes considered to be a term encompassing Besnier prurigo and other conditions.
It is sometimes considered a diagnosis of exclusion.
Venous eczema (gravitational eczema, stasis dermatitis, varicose eczema) occurs in people with impaired circulation, varicose veins, and edema, and is particularly common in the ankle area of people over 50. There is redness, scaling, darkening of the skin, and itching. The disorder predisposes to leg ulcers. (ICD-10 I83.1)