List Of Cutaneous (Skin) Conditions - Papulosquamous Hyperkeratotic

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List Of Cutaneous (Skin) Conditions - Papulosquamous Hyperkeratotic

There are many conditions of or affecting the human integumentary system—the organ system that covers the entire surface of the body and is 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. There are two main types of human skin: glabrous skin, the non-hairy skin on the palms and soles (also referred to as the "palmoplantar" surfaces), and hair-bearing skin. Within the latter type, there are hairs 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 via diffusion from the dermis, since the epidermis is without direct blood supply. The epidermis contains four cell types: keratinocytes, melanocytes, Langerhans cells, and the Merkel cell. 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; it takes about two weeks 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 and reticular dermis. The superficial papillary dermis interdigitates 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 extrafibrillar matrix (previously called 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 colour (red, blue, brown, black, white, yellow). The diagnosis of a many conditions often also requires a skin biopsy which yields histologic information that can be correlated with the clinical presentation and any laboratory data.

 

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Diseases – Cutaneous (Skin) Diseases – Papulosquamous Hyperkeratotic

 

Papulosquamous hyperkeratotic cutaneous conditions are those that present with papules and scales caused by a thickening of the stratum corneum.

 

 

Palmoplantar keratodermas

 

Palmoplantar keratodermas are a diverse group of hereditary and acquired keratodermas in which there is hyperkeratosis of the skin of the palms and soles.

 

  • Acrokeratoelastoidosis of Costa (Keratoelastoidosis marginalis)
  • Aquagenic keratoderma (Acquired aquagenic palmoplantar keratoderma, Aquagenic syringeal acrokeratoderma, Aquagenic wrinkling of the palms, Transient reactive papulotranslucent acrokeratoderma)
  • Bart–Pumphrey syndrome (Palmoplantar keratoderma with knuckle pads and leukonychia and deafness)
  • Camisa disease
  • Carvajal syndrome (Striate palmoplantar keratoderma with woolly hair and cardiomyopathy, Striate palmoplantar keratoderma with woolly hair and left ventricular dilated cardiomyopathy)
  • Corneodermatoosseous syndrome (CDO syndrome)
  • Diffuse epidermolytic palmoplantar keratoderma (Palmoplantar keratoderma cum degeneratione granulosa Vörner, Vörner's epidermolytic palmoplantar keratoderma, Vörner keratoderma)
  • Diffuse nonepidermolytic palmoplantar keratoderma (Diffuse orthohyperkeratotic keratoderma, Hereditary palmoplantar keratoderma, Keratosis extremitatum progrediens, Keratosis palmoplantaris diffusa circumscripta, Tylosis, Unna–Thost disease, Unna–Thost keratoderma)
  • Erythrokeratodermia variabilis (Erythrokeratodermia figurata variabilis, Keratosis extremitatum progrediens, Keratosis palmoplantaris transgrediens et progrediens, Mendes da Costa syndrome, Mendes da Costa type erythrokeratodermia, Progressive symmetric erythrokeratoderma)
  • Focal acral hyperkeratosis (Acrokeratoelastoidosis lichenoides, Degenerative collagenous plaques of the hand)
  • Focal palmoplantar and gingival keratosis
  • Focal palmoplantar keratoderma with oral mucosal hyperkeratosis (Focal epidermolytic palmoplantar keratoderma, Hereditary painful callosities, Hereditary painful callosity syndrome, Keratosis follicularis, Keratosis palmoplantaris nummularis, Nummular epidermolytic palmoplantar keratoderma)
  • Haim–Munk syndrome (Palmoplantar keratoderma with periodontitis and arachnodactyly and acro-osteolysis)
  • Hidrotic ectodermal dysplasia (Alopecia congenita with keratosis palmoplantaris, Clouston syndrome, Clouston's hidrotic ectodermal dysplasia, Fischer–Jacobsen–Clouston syndrome, Keratosis palmaris with drumstick fingers, Palmoplantar keratoderma and clubbing)
  • Howel–Evans syndrome (Familial keratoderma with carcinoma of the esophagus, Focal non-epidermolytic palmoplantar keratoderma with carcinoma of the esophagus, Palmoplantar ectodermal dysplasia type III, Palmoplantar keratoderma associated with esophageal cancer, Tylosis, Tylosis–esophageal carcinoma)
  • Hystrix-like ichthyosis–deafness syndrome (HID syndrome)
  • Keratoderma climactericum (Acquired plantar keratoderma, Climacteric keratoderma, Haxthausen's disease)
  • Keratosis punctata palmaris et plantaris (Buschke–Fischer–Brauer disease, Davis Colley disease, Keratoderma disseminatum palmaris et plantaris, Keratosis papulosa, Keratoderma punctatum, Keratodermia punctata, Keratoma hereditarium dissipatum palmare et plantare, Palmar and plantar seed dermatoses, Palmar keratoses, Papulotranslucent acrokeratoderma, Punctate keratoderma, Punctate keratoses of the palms and soles, Maculosa disseminata)
  • Keratitis–ichthyosis–deafness syndrome (Erythrokeratodermia progressiva Burns, Ichthyosiform erythroderma with corneal involvement and deafness, KID syndrome)
  • Mal de Meleda (Acral keratoderma, Mutilating palmoplantar keratoderma of the Gamborg–Nielsen type, Palmoplantar ectodermal dysplasia type VIII, Palmoplantar keratoderma of the Norrbotten type)
  • Naxos syndrome (Diffuse non-epidermolytic palmoplantar keratoderma with woolly hair and cardiomyopathy, Diffuse palmoplantar keratoderma with woolly hair and arrythmogenic right ventricular cardiomyopathy of Naxos, Naxos disease)
  • Olmsted syndrome (Mutilating palmoplantar keratoderma with periorificial keratotic plaques, Mutilating palmoplantar keratoderma with periorificial plaques, Polykeratosis of Touraine)
  • Pachyonychia congenita type I (Jadassohn–Lewandowsky syndrome)
  • Pachyonychia congenita type II (Jackson–Lawler pachyonychia congenita, Jackson–Sertoli syndrome)
  • Palmoplantar keratoderma and spastic paraplegia (Charcot–Marie–Tooth disease with palmoplantar keratoderma and nail dystrophy)
  • Palmoplantar keratoderma of Sybert (Greither palmoplantar keratoderma, Greither syndrome, Keratosis extremitatum hereditaria progrediens, Keratosis palmoplantaris transgrediens et progrediens, Sybert keratoderma, Transgrediens and progrediens palmoplantar keratoderma)
  • Papillon–Lefèvre syndrome (Palmoplantar keratoderma with periodontitis)
  • Porokeratosis plantaris discreta
  • Punctate palmoplantar keratoderma
  • Schöpf–Schulz–Passarge syndrome (Eyelid cysts with palmoplantar keratoderma and hypodontia and hypotrichosis)
  • Scleroatrophic syndrome of Huriez (Huriez syndrome, Palmoplantar keratoderma with scleroatrophy, Palmoplantar keratoderma with sclerodactyly, Scleroatrophic and keratotic dermatosis of the limbs, Sclerotylosis)
  • Striate palmoplantar keratoderma (Acral keratoderma, Brünauer–Fuhs–Siemens type of palmoplantar keratoderma, Focal non-epidermolytic palmoplantar keratoderma, Keratosis palmoplantaris varians, Palmoplantar keratoderma areata, Palmoplantar keratoderma striata, Wachter keratoderma, Wachters palmoplantar keratoderma)
  • Spiny keratoderma (Porokeratosis punctata palmaris et plantaris, Punctate keratoderma, Punctate porokeratosis of the palms and soles)
  • Tyrosinemia type II (Oculocutaneous tyrosinemia, Richner–Hanhart syndrome)
  • Vohwinkel syndrome (Keratoderma hereditaria mutilans, Keratoma hereditaria mutilans, Mutilating keratoderma of Vohwinkel, Mutilating palmoplantar keratoderma)
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