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Keratoacanthoma usually occurs in older individuals. As with squamous cell cancer, it seems likely that ultraviolet light from the sun causes the development of KA. As with squamous cell cancer, sporadic cases have been found co-infected with the human papilloma virus (HPV).
Many new treatments for Melanoma are also known to increase the rate of Keratoacanthoma, such as the B-Raf inhibitor drugs Vemurafenib and Dabrafenib.
Actinic keratosis is very common, with an estimated 14% of dermatology visits related to AKs. It is seen more often in fair-skinned individuals, and rates vary with geographical location and age. Other factors such as exposure to ultraviolet (UV) radiation, certain phenotypic features, and immunosuppression can also contribute to the development of AKs.
Men are more likely to develop AK than women, and the risk of developing AK lesions increases with age. These findings have been observed in multiple studies, with numbers from one study suggesting that approximately 5% of women ages 20–29 develop AK compared to 68% of women ages 60–69, and 10% of men ages 20–29 develop AK compared to 79% of men ages 60–69.
Geography seems to play a role in the sense that individuals living in locations where they are exposed to more UV radiation throughout their lifetime have a significantly higher risk of developing AK. Much of the literature on AK comes from Australia, where prevalence of AK is estimated at 40–50% in adults over 40, as compared to the United States and Europe, where prevalence is estimated at under 11–38% in adults. One study found that those who immigrated to Australia after age 20 had fewer AKs than native Australians in all age groups.
Keratoacanthomas (molluscum sebaceum) may be divided into the following types:
- "Giant keratoacanthomas" are a variant of keratoacanthoma, which may reach dimensions of several centimeters.
- "Keratoacanthoma centrifugum marginatum" is a cutaneous condition, a variant of keratoacanthomas, which is characterized by multiple tumors growing in a localized area.
- "Multiple keratoacanthomas" (also known as "Ferguson–Smith syndrome," "Ferguson-Smith type of multiple self-healing keratoacanthomas,") is a cutaneous condition, a variant of keratoacanthomas, which is characterized by the appearance of multiple, sometimes hundreds of keratoacanthomas.
- A "solitary keratoacanthoma" (also known as "Subungual keratoacanthoma") is a benign, but rapidly growing, locally aggressive tumor which sometimes occur in the nail apparatus.
- "Generalized eruptive keratoacanthoma" (also known as "Generalized eruptive keratoacanthoma of Grzybowski") is a cutaneous condition, a variant of keratoacanthomas, characterized by hundreds to thousands of tiny follicular keratotic papules over the entire body. Treatments are not successful for many patients with Generalized eruptive keratoacanthoma. Use of emollients and anti-itch medications can ease some symptoms. Improvement or complete resolutions of the condition has occurred with the application of the following medications: Acitretin, Isotretinoin, Fluorouracil, Methotrexate, Cyclophosphamide.
Solitary cutaneous leiomyoma typically presents as a deeply circumscribed, freely movable, rounded nodule ranging from 2 to 15mm in diameter, with overlying skin that may have a reddish or violaceous tint.
AKs follow one of three paths: they can either persist as AKs, regress, or progress to invasive skin cancer, as AK lesions are considered to be on the same continuum with squamous cell carcinoma (SCC). AK lesions that regress also have the potential to recur.
- Progression: The overall risk of an AK turning into invasive cancer is low. In average-risk individuals, likelihood of an AK lesion progressing to SCC is less than 1% per year. Despite this low rate of progression, studies suggest that a full 60% of SCCs arise from pre-existing AKs, reinforcing the idea that these lesions are closely related.
- Regression: Reported regression rates for single AK lesions have ranged between 15–63% after one year.
- Recurrence: Recurrence rates after 1 year for single AK lesions that have regressed range between 15–53%.
A 2006 review stated that RS often leads renal cancer between ages 30-50. Renal cancer kills about 1 in 3 people, but 5-year survival rates improved between 1974-1976 and 1995-2000, from 52% to 64%.
The etiology of florid cutaneous papillomatosis is unknown. It is likely directly induced by an underlying neoplasm secreting a growth factor. One candidate may be alpha-transforming growth factor, structurally related to epidermal growth factor, but antigenically distinct from it. The underlying cancer is most often gastric adenocarcinoma but also with breast cancer, bladder cancer, hepatobiliary cancer, ovarian cancer, uterine cancer, prostate cancer, lung cancer and cervical cancer. Other associated underlying malignancies include squamous cell carcinomas and lymphomas.
In dermatologic pathology, a dermal cylindroma, also dermal eccrine cylindroma or Cutaneous Cylindroma) and (less specifically) cylindroma, is a benign adnexal tumor, which occurs on the scalp and forehead.when multiple Cylindroma-Show Hat like configration.
Multiple cylindromas may grow together in a "hat-like" configuration, sometimes referred to as a turban tumor. Cylindromas are uncommon dysplasias of skin appendages.
Florid cutaneous papillomatosis, malignant acanthosis nigricans, and the sign of Leser-Trélat may be highlighted as part of a continuum, with each having a common or similar pathogenic pathway due to an underlying malignancy that produces a factor epidermal growth factor-like activity.
Multiple cutaneous leiomyomas, also known as Pilar leiomyomas, arise from the arrectores pilorum muscles, and are made up of a poorly circumscribed proliferation of haphazardly arranged smooth muscle fibers located in the dermis that appear to infiltrate the surrounding tissue and may extend into the subcutis.
Sometimes associated with uterine leiomyomas (a combination known as multiple cutaneous and uterine leiomyomatosis, MCUL), these lesions may also be a manifestation of the hereditary leiomyomatosis and renal cell cancer syndrome.
Benign metastasizing leiomyoma is a rare condition characterized by the growth of uterine leiomyoma in the other regions especially the lungs.
Dermal cylindromas are:
- dermal lesions consisting of nests of cells that are surrounded by hyaline (i.e. glassy, eosinophilic, acellular) material and have:
- hyperchromatic nuclei that may palisade (columnar nuclei arranged around the periphery of the cells nests - with their short axis tangential to the nest periphery), and
- cells with lighter staining ovoid nuclei at their centre.
They lack of a significant number of lymphocytes; this differentiates them from spiradenomas.
Mesenchymal neoplasms of the gallbladder are rare and in particular leiomyomas of the gallbladder have been rarely reported, all of them in patients with immune system disorders. Although, recently, a case was reported in absence of associated immunodeficiency at Monash Hospital in Melbourne Australia in a healthy 39-year-old woman with no symptoms.
Other relatively rare conditions have been reported in association with this disease. It is not yet known if these associations are fortuitous or manifestations of the condition itself.
Cerebral cavernomas and massive, macronodular adrenocortical disease have also been reported in association with this syndrome. A case of cutis verticis gyrata, disseminated collagenoma and Charcot-Marie-Tooth disease in association with a mutation in the fumarate hydratase gene has also been reported. Two cases of ovarian mucinous cystadenoma have also been reported with this mutation.
Leiomyoma is the most common benign mesenchymal tumor of esophagus and second most common benign tumor of the small bowel (with gastrointestinal stromal tumor as most common). Although leiomyoma is the most common benign esophageal tumor, malignant carcinoma is still 50 times more likely. Approximately 50% of cases are found in the jejunum, followed by the ileum in 31% of cases. Almost one half of all lesions are less than 5 centimeters.
Infantile hemangiopericytoma (also known as "Congenital hemangiopericytoma") is a cutaneous condition characterized by single or multiple dermal and subcutaneous nodules that may be alarmingly large at birth or grow rapidly.
Trichilemmoma (also known as "tricholemmoma") is a benign cutaneous neoplasm that shows differentiation toward cells of the outer root sheath. The lesion is often seen in the face and neck region. Multifocal occurrence is associated with Cowden syndrome, in which hamartomatous intestinal polyposis is seen in conjunction with multiple tricholemmoma lesions.
Angioleiomyoma (vascular leiomyoma, angiomyoma) of the skin is thought to arise from vascular smooth muscle, and is generally acquired.
Benign neonatal hemangiomatosis is a cutaneous condition in infants, characterized by multiple cutaneous lesions without evident visceral hemangiomas.
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.
Screening for melanoma in FAMMM kindreds should begin at age 10 with a baseline total body skin examination including scalp, eyes, oral mucosa, genital area, and nail, as family members may develop melanoma in their early teens.
At Mayo Clinic, FAMMM patients with a confirmed mutation and family history of pancreatic cancer are offered screening with either high-resolution pancreatic protocol CT, MRI, or endoscopic ultrasound starting at age 50 or 10 years younger than the earliest family member with pancreas cancer. They are counseled on the lack of evidence-based data to support screening, and on the limitations of our current technology to detect a lesion at a stage amenable to therapy.
Drug-induced lipodystrophy is a cutaneous condition that presents as one or multiple depressed areas (i.e. indentations), usually on the proximal extremities, ranging from under a few centimeters to greater than 20 cm in diameter.
Nodular lichen myxedematosus is a cutaneous condition characterized by multiple nodules on the limbs and trunk, with a mild or absent papular component.
Birt-Hogg-Dubé Syndrome patients, families, and caregivers are encouraged to join the NIH Rare Lung Diseases Consortium Contact Registry. This is a privacy protected site that provides up-to-date information for individuals interested in the latest scientific news, trials, and treatments related to rare lung diseases.
Blastomycosis-like pyoderma (also known as "Pyoderma vegetans") is a cutaneous condition characterized by large verrucous plaques with elevated borders and multiple pustules.