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Clinically, three distinct patterns of palmoplantar keratoderma may be identified: diffuse, focal, and punctate.
Diffuse palmoplantar keratoderma is a type of palmoplantar keratoderma that is characterized by an even, thick, symmetric hyperkeratosis over the whole of the palm and sole, usually evident at birth or in the first few months of life. Restated, diffuse palmoplantar keratoderma is an autosomal dominant disorder in which hyperkeratosis is confined to the palms and soles. The two major types can have a similar clinical appearance:
- "Diffuse epidermolytic palmoplantar keratoderma" (also known as "Palmoplantar keratoderma cum degeneratione granulosa Vörner," "Vörner's epidermolytic palmoplantar keratoderma", and "Vörner keratoderma") is one of the most common patterns of palmoplantar keratoderma, an autosomal dominant condition that presents within the first few months of life, characterized by a well-demarcated, symmetric thickening of palms and soles, often with a "dirty" snakeskin appearance due to underlying epidermolysis.
- "Diffuse nonepidermolytic palmoplantar keratoderma" (also known as "Diffuse orthohyperkeratotic keratoderma," "Hereditary palmoplantar keratoderma," "Keratosis extremitatum progrediens," "Keratosis palmoplantaris diffusa circumscripta," "Tylosis," "Unna–Thost disease", and "Unna–Thost keratoderma") is inherited as an autosomal dominant condition and is present from infancy, characterized by a well-demarcated, symmetric, often "waxy" keratoderma involving the whole of the palms and soles.
Hidrotic ectodermal dysplasia 2, or Clouston syndrome (referred to as HED2 throughout this entry) is characterized by partial or total alopecia, dystrophy of the nails, hyperpigmentation of the skin (especially over the joints), and clubbing of the fingers. Sparse scalp hair and dysplastic nails are seen early in life. In infancy, scalp hair is wiry, brittle, patchy, and pale; progressive hair loss may lead to total alopecia by puberty. The nails may be milky white in early childhood; they gradually become dystrophic, thick, and distally separated from the nail bed. Palmoplantar keratoderma may develop during childhood and increases in severity with age. The clinical manifestations are highly variable even within the same family.
Reticulated seborrheic keratosis (also known as "Adenoid seborrheic keratosis") is a common benign cutaneous condition characterized by a skin lesion with a dull or lackluster surface, and with keratin cysts seen histologically.
Ectodermal dysplasia with corkscrew hairs is a skin condition with salient features including exaggerated pili torti, scalp keloids, follicular plugging, keratosis pilaris, xerosis, eczema, palmoplantar keratoderma, syndactyly, onchodysplasia, and conjunctival neovascularization.
Seborrheic keratoses may be divided into the following types:
- Common seborrheic keratosis (basal cell papilloma, solid seborrheic keratosis)
- Reticulated seborrheic keratosis (adenoid seborrheic keratosis)
- Stucco keratosis (digitate seborrheic keratosis, hyperkeratotic seborrheic keratosis, serrated seborrheic keratosis, verrucous seborrheic keratosis) -- Often are light brown to off-white. Pinpoint to a few millimeters in size. Often found on the distal tibia, ankle, and foot.
- Clonal seborrheic keratosis
- Irritated seborrheic keratosis (inflamed seborrheic keratosis)
- Seborrheic keratosis with squamous atypia
- Melanoacanthoma (pigmented seborrheic keratosis)
- Dermatosis papulosa nigra—Commonly found among adult dark-skinned individuals, presents on the face as small benign papules from a pinpoint to a few millimeters in size.
- Inverted follicular keratosis
Also see:
- The sign of Leser-Trélat
A seborrheic keratosis, also known as seborrheic verruca, basal cell papilloma, or a senile wart, is a non-cancerous (benign) skin tumour that originates from cells in the outer layer of the skin (keratinocytes). Like liver spots, seborrheic keratoses are seen more often as people age.
The tumours (also called lesions) appear in various colours, from light tan to black. They are round or oval, feel flat or slightly elevated, like the scab from a healing wound, and range in size from very small to more than across. They can often come in association with other skin conditions, including basal cell carcinoma. Sometimes seborrheic keratosis and basal cell carcinoma occur at the same location, and sometimes seborrheic keratosis progresses to basal cell carcinoma. At clinical examination the differential diagnosis include warts and melanoma. Because only the top layers of the epidermis are involved, seborrheic keratoses are often described as having a "pasted on" appearance. Some dermatologists refer to seborrheic keratoses as "seborrheic warts", because they resemble warts, but strictly speaking the term "warts" refers to lesions that are caused by human papillomavirus.
Warty dyskeratoma must be differentiated from vulvar dysplasia, Bowenoid papulosis, squamous carcinoma, condyloma, and other viral-induced squamous lesions.
Warty dyskeratoma, also known as an Isolated dyskeratosis follicularis, is a benign epidermal proliferation with distinctive histologic findings that may mimic invasive squamous cell carcinoma and commonly manifests as an umbilicated (Having a central mark or depression resembling a navel) lesion with a keratotic plug, WD have some histopathologic similarities to viral warts but it's not caused by HPV and the majority of these lesions display overall histopathologic features consistent with a follicular adnexal neoplasm. usually limited to the head, neck, scalp or face and vulva. Lesions are generally and sporadic and may be associated with a follicular unit. Oral involvement, particularly the hard palate, and genital involvement have been reported. it can also be thought of as one of the manifestations of focal acantholytic dyskeratosis, an epidermal reaction pattern that can be seen in several disorders, including Darier's disease and Grover's disease. But the main Difference between Darier disease and Warty dyskeratoma, is that Darier disease inherited dermatosis (autosomal dominant) consisting of multiple keratotic papules on the face, trunk, and extremities, while WD occurs as an isolated, noninherited, single keratotic nodule mainly confined to the head and neck as mentioned earlier.
Clonal seborrheic keratosis is a common benign cutaneous condition characterized by a skin lesion with a dull or lackluster surface, and with round, loosely packed nests of cells seen histologically.
Keratosis follicularis spinulosa decalvans (also known as "Siemens-1 syndrome") is a rare X-linked disorder described by Siemens in 1926, a disease that begins in infancy with keratosis pilaris localized on the face, then evolves to more diffuse involvement.
An association with SAT1 has been suggested.
Meleda disease (MDM) or "mal de Meleda", also called Mljet disease, keratosis palmoplantaris and transgradiens of Siemens, (also known as "Acral keratoderma," "Mutilating palmoplantar keratoderma of the Gamborg-Nielsen type," "Palmoplantar ectodermal dysplasia type VIII", and "Palmoplantar keratoderma of the Norrbotten type") is an extremely rare autosomal recessive congenital skin disorder in which dry, thick patches of skin develop on the soles of the hands and feet, a condition known as palmoplantar hyperkeratosis.
A stucco keratosis is a common benign skin condition characterized by a lesion with a dull or lackluster surface, and with church-spire-like projections of epidermal cells around collagen seen histologically. Stucco keratoses are often light brown to off-white, and are no larger than a few millimeters in diameter. They are often found on the distal tibia, ankle, and foot.
Other names for a stucco keratosis are "digitate seborrheic keratosis," "dyperkeratotic seborrheic keratosis," "deratosis alba," "serrated seborrheic keratosis," and "verrucous seborrheic keratosis".
Focal palmoplantar and gingival keratosis is a rare autosomal dominant disease whose clinical features, and in particular, pathologic alterations and molecular mechanisms remains to be well defined.
Common seborrheic keratosis (also known as "Basal cell papilloma," and "Solid seborrheic keratosis") is a common benign cutaneous condition characterized by a skin lesion with a dull or lackluster surface.
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.
The most common location by far is the gingival margin and other areas of the masticatory oral mucosa, these occur more frequently in the fifth decade of life, and have good prognosis, the treatment of choice for oral VXs is surgical excision, and recurrence is rare.
The condition can affect other organs of body, such as the penis, vulva, and can occur in anal region, nose, the ear, lower extremity, scrotum.
Seborrheic keratosis with squamous atypia is a less common cutaneous condition characterized by a skin lesion with a dull or lackluster surface, and with round, loosely packed nests of cells seen histologically.
Erythrokeratodermia variabilis (also known as "erythrokeratodermia figurata variabilis", "keratosis extremitatum progrediens", "keratosis palmoplantaris transgrediens et progrediens", "Mendes da Costa syndrome", "Mendes da Costa type erythrokeratodermia", and "progressive symmetric erythrokeratoderma") is a rare autosomal dominant disorder that usually presents at birth or during the first year of life. To date, it is thought to be caused by mutations in genes encoding for connexin channels proteins in the epidermis, leading to the misregulation of homeostasis in keratinocytes.
One type is characterized by generalized, persistent, brown hyperkeratosis with accentuated skin markings, while a second type is localized, with involvement that is limited in extent and characterized by sharply demarcated, hyperkeratotic plaques.
It can be associated with GJB3 and GJB4.
It was characterized in 1925.
Irritated seborrheic keratosis (also known as "Basosquamous cell acanthoma," and "Inflamed seborrheic keratosis") is a common benign cutaneous condition characterized by a skin lesion with a dull or lackluster surface.
Microscopically, inverted follicular keratosis is characterized as a well-circumscribed inverted acanthotic squamous proliferation containing squamous eddies and without significant atypia.
The presentation may be of alopecia (baldness). Individuals vary in severity of symptoms. Nail deformities may also be present as well as hair follicle keratosis and follicular hyperkeratosis.
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.
Melanoacanthoma (also known as "Pigmented seborrheic keratosis") is a common, benign, darkly pigmented cutaneous condition characterized by a skin lesion with a dull or lackluster surface.
It involves a proliferation of keratinocytes and melanocytes.
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).