Multiple minute digitate hyperkeratosis | |
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Specialty | Dermatology |
Multiple minute digitate hyperkeratosis, also known as digitate keratoses, disseminated spiked hyperkeratosis, familial disseminated piliform hyperkeratosis, and minute aggregate keratosis is a rare cutaneous condition, with about half of cases being familial, inherited in an autosomal dominant fashion, while the other half are sporadic. [1]
Multiple minute digitate hyperkeratosis is mostly a nonfollicular digitate keratosis that affects the limbs and trunk. There is no impact on the face or palmoplantar surfaces. The lesions consist of skin-colored, yellow, brown, or white spicules that range in length from 0.5 to 5.0 mm and diameter from 0.3 to 3.0 mm. Occasionally, flat-topped, dome-shaped, or crateriform papules are also present. [2]
Multiple minute digitate hyperkeratosis may be sporadic or familial. [2]
Histopathology reveals a stratum granulosum with varying thickness and localized orthokeratotic hyperkeratosis originating from a tented epidermis. Rarely, there have also been reports of superficial cutaneous lymphoplasmocytic infiltrates, parakeratosis, and epidermal invagination. A recurring characteristic that unites all of the cases that have been documented is the absence of follicular involvement. [3]
Reduced keratohyaline granules with varying numbers of Odland bodies are seen under electron microscopy. [3]
While improvements with various topical keratolytics and topical and oral retinoids have been reported, treatment is frequently ineffective or only temporary. [2]
Cutaneous squamous-cell carcinoma (cSCC), also known as squamous-cell carcinoma of the skin or squamous-cell skin cancer, is one of the three principal types of skin cancer, alongside basal-cell carcinoma and melanoma. cSCC typically presents as a hard lump with a scaly surface, though it may also present as an ulcer. Onset and development often occurs over several months. Compared to basal cell carcinoma, cSCC is more likely to spread to distant areas. When confined to the epidermis, the outermost layer of the skin, the pre-invasive or in situ form of cSCC is termed Bowen's disease.
A skin condition, also known as cutaneous condition, is any medical condition that affects the integumentary system—the organ system that encloses the body and includes skin, nails, and related muscle and glands. The major function of this system is as a barrier against the external environment.
Lichen planus (LP) is a chronic inflammatory and autoimmune disease that affects the skin, nails, hair, and mucous membranes. It is not an actual lichen, but is named for its appearance. It is characterized by polygonal, flat-topped, violaceous papules and plaques with overlying, reticulated, fine white scale, commonly affecting dorsal hands, flexural wrists and forearms, trunk, anterior lower legs and oral mucosa. The hue may be gray-brown in people with darker skin. Although there is a broad clinical range of LP manifestations, the skin and oral cavity remain as the major sites of involvement. The cause is unknown, but it is thought to be the result of an autoimmune process with an unknown initial trigger. There is no cure, but many different medications and procedures have been used in efforts to control the symptoms.
Darier's disease (DAR) is a rare, inherited skin disorder that presents with multiple greasy, crusting, thick brown bumps that merge into patches. It is an autosomal dominant disorder discovered by French dermatologist Ferdinand-Jean Darier.
A seborrheic keratosis is a non-cancerous (benign) skin tumour that originates from cells, namely keratinocytes, in the outer layer of the skin called the epidermis. Like liver spots, seborrheic keratoses are seen more often as people age.
Actinic keratosis (AK), sometimes called solar keratosis or senile keratosis, is a pre-cancerous area of thick, scaly, or crusty skin. Actinic keratosis is a disorder of epidermal keratinocytes that is induced by ultraviolet (UV) light exposure. These growths are more common in fair-skinned people and those who are frequently in the sun. They are believed to form when skin gets damaged by UV radiation from the sun or indoor tanning beds, usually over the course of decades. Given their pre-cancerous nature, if left untreated, they may turn into a type of skin cancer called squamous cell carcinoma. Untreated lesions have up to a 20% risk of progression to squamous cell carcinoma, so treatment by a dermatologist is recommended.
Palmoplantar keratodermas are a heterogeneous group of disorders characterized by abnormal thickening of the stratum corneum of the palms and soles.
Hyperkeratosis is thickening of the stratum corneum, often associated with the presence of an abnormal quantity of keratin, and is usually accompanied by an increase in the granular layer. As the corneum layer normally varies greatly in thickness in different sites, some experience is needed to assess minor degrees of hyperkeratosis.
Discoid lupus erythematosus is the most common type of chronic cutaneous lupus (CCLE), an autoimmune skin condition on the lupus erythematosus spectrum of illnesses. It presents with red, painful, inflamed and coin-shaped patches of skin with a scaly and crusty appearance, most often on the scalp, cheeks, and ears. Hair loss may occur if the lesions are on the scalp. The lesions can then develop severe scarring, and the centre areas may appear lighter in color with a rim darker than the normal skin. These lesions can last for years without treatment.
Florid cutaneous papillomatosis (FCP), is an obligate paraneoplastic syndrome.
Chilblain lupus erythematosus was initially described by Hutchinson in 1888 as an uncommon manifestation of chronic cutaneous lupus erythematosus. Chilblain lupus erythematosus is characterized by a rash that primarily affects acral surfaces that are frequently exposed to cold temperatures, such as the toes, fingers, ears, and nose. The rash is defined by oedematous skin, nodules, and tender plaques with a purple discoloration.
Acrokeratoelastoidosis of Costa or Acrokeratoelastoidosis is a hereditary form of marginal keratoderma, and can be defined as a palmoplantar keratoderma. It is distinguished by tiny, firm pearly or warty papules on the sides of the hands and, occasionally, the feet. It is less common than the hereditary type of marginal keratoderma, keratoelastoidosis marginalis.
Erythromelanosis follicularis faciei et colli is an erythematous pigmentary disease involving the follicles, characterized by a reddish-brown, sharply demarcated, symmetrical discoloration involving the preauricular and maxillary regions.
Traumatic anserine folliculosis is a curious gooseflesh-like follicular hyperkeratosis that may result from persistent pressure and lateral friction of one skin surface against another. Traumatic anserine folliculosis is caused by trauma. Topical keratolytics are the treatment of choice.
An arsenical keratosis is a growth of keratin on the skin caused by arsenic, which occurs naturally in the Earth's crust and is widely distributed in the environment, Arsenical compounds are used in industrial, agricultural, and medicinal substances. Arsenic is also found to be an environmental contaminant in drinking water and an occupational hazard for miners and glass workers. Arsenic may also causes other conditions including: Bowen's disease, cardiovascular diseases, developmental abnormalities, neurologic and neurobehavioral disorders, diabetes, hearing loss, hematologic disorders, and various types of cancer. Arsenical keratoses may persist indefinitely, and some may develop into invasive squamous cell carcinoma. Metastatic arsenic squamous cell carcinoma and arsenic-induced malignancies in internal organs such as the bladder, kidney, skin, liver, and colon, may result in death.
Acrokeratosis verruciformis is a rare autosomal dominant disorder appearing at birth or in early childhood, characterized by skin lesions that are small, verrucous, flat papules resembling warts along with palmoplantar punctate keratoses and pits. However sporadic forms, whose less than 10 cases have been reported, presents at a later age, usually after the first decade and generally lack palmoplantar keratoses. Whether acrokeratosis verruciformis and Darier disease are related or distinct entities has been controversial, like Darier's disease, it is associated with defects in the ATP2A2 gene. however the specific mutations found in the ATP2A2 gene in acrokeratosis verruciformis have never been found in Darier's disease.
Reticular erythematous mucinosis (REM) is a skin condition caused by fibroblasts producing abnormally large amounts of mucopolysaccharides. It is a disease that tends to affect women in the third and fourth decades of life.
Trichodysplasia spinulosa is a rare cutaneous condition that has been described almost exclusively in immunocompromised patients, usually organ transplant recipients, on regimens of immunosuppressive drugs. As of early 2016, a total of 32 cases had been reported in the medical literature. Despite its rarity, TS is believed to be underdiagnosed, and the growing population of patients on immunosuppressive drug regimens suggests its incidence may rise. TS has been described as an emerging infectious disease.
Rheumatoid neutrophilic dermatitis, also known as rheumatoid neutrophilic dermatosis, is a cutaneous condition associated with rheumatoid arthritis.