Palmoplantar keratodermas are a heterogeneous group of skin disorders characterized by abnormal thickening (scleroderma) of the stratum corneum of the palms and soles.
Sclerodactyly is a localized thickening and tightness of the skin of the fingers or toes that yields a characteristic claw-like appearance and spindle shape of the affected digits, and renders them immobile or of limited mobility. The thickened, discolored patches of skin are called morphea, and may involve connective tissue below the skin, as well as muscle and other tissues. Sclerodactyly is often preceded by months or even years by Raynaud's phenomenon when it is part of systemic scleroderma.
Meleda disease (MDM) or "mal de Meleda", also called Mljet disease, keratosis palmoplantaris and transgradiens of Siemens, 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. Meleda Disease is a skin condition which usually can be identified not long after birth. This is a genetic condition but it is very rare. The hands and feet usually are the first to show signs of the disease but the disease can advance to other parts of the body. Signs of the disease include thickening of the skin, on hands and soles of feet, which can turn red in color. There currently is no cure and treatment is limited, but Acitretin can be used in severe cases.
Pruritic urticarial papules and plaques of pregnancy (PUPPP), known in the United Kingdom as polymorphic eruption of pregnancy (PEP), is a chronic hives-like rash that strikes some women during pregnancy. Some skin changes are known to occur in people who are pregnant while other skin conditions, or dermatoses, that people have prior to getting pregnant will become altered or symptoms will increase. Pruritic urticarial papules and plaques of pregnancy (PUPPP) is one of many skin conditions that is specific to pregnancy and occurs in about 1 in every 160 (0.625%) of pregnancies.
Dermatopathia pigmentosa reticularis(DPR) is a rare, autosomal dominant congenital disorder that is a form of ectodermal dysplasia. Dermatopathia pigmentosa reticularis is composed of the triad of generalized reticulate hyperpigmentation, noncicatricial alopecia, and onychodystrophy. DPR is a non life-threatening disease that largely affects the skin, hair, and nails. It has also been identified as a keratin disorder. Historically, as of 1992, only 10 cases had been described in world literature; however, due to recent advances in genetic analysis, five additional families studied in 2006 have been added to the short list of confirmed cases.
Lichen nitidus is a chronic inflammatory disease of unknown cause characterized by 1–2 mm, discrete and uniform, shiny, flat-topped, pale flesh-colored or reddish-brown papules that may appear as hypopigmented against dark skin. Occasionally, minimal scaling is present or can be induced by rubbing the surface of the papules. The disease usually affects children and young adults and is painless and usually nonpruritic, although protracted itching may occur in some cases. It is sometimes referred to by dermatologists as "mini lichen planus".
Aquagenic urticaria, also known as water allergy and water urticaria, is a form of physical urticaria in which hives develop on the skin after contact with water, regardless of its temperature. The condition typically results from contact with water of any type, temperature or additive.
Anetoderma is a benign but uncommon disorder that causes localized areas of flaccid or herniated sac-like skin due to a focal reduction of dermal elastic tissue. Anetoderma is subclassified as primary anetoderma, secondary anetoderma, iatrogenic anetoderma of prematurity, congenital anetoderma, familial anetoderma, and drug-induced anetoderma.
Kyrle disease is identified as a form of an acquired perforating disease. Other major perforating diseases are elastosis perforans serpiginosa and reactive perforating collagenosis. Recently, however, there is a controversy on categorizing Kyrle disease with perforating dermatosis or a subtype of acquired perforating collagenosis.
Knuckle pads, also known as heloderma, meaning similar to the skin of the Gila monster lizard for which it is named, are circumscribed, keratotic, fibrous growths over the dorsa of the interphalangeal joints. They are described as well-defined, round, plaque-like, fibrous thickening that may develop at any age, and grow to be 10 to 15mm in diameter in the course of a few weeks or months, then go away over time.
Targetoid hemosiderotic hemangioma, also known as a hobnail hemangioma is a skin condition characterized by a central brown or purplish papule that is surrounded by an ecchymotic halo. It may appear similar to melanoma. It was first described by Santa Cruz and Aronberg in 1988.
Recurrent palmoplantar hidradenitis, also known as idiopathic palmoplantar hidradenitis, idiopathic plantar hidradenitis, painful plantar erythema, palmoplantar eccrine hidradenitis, and plantar panniculitis, is primarily a disorder of healthy children and young adults, characterized by lesions that are primarily painful, subcutaneous nodules on the plantar surface, resembling erythema nodosum.
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.
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.
Acral persistent papular mucinosis (APPM) is a rare form of lichen myxedematosus. It is characterized by small papules on the backs of the hands, wrists, and extensor aspects of the distal forearms, with no further clinical or laboratory indications. Lesions tend to persist and may grow in number gradually. Because there are no symptoms, treatment is rarely required.
Generalized granuloma annulare is a skin condition of unknown cause, tending to affect women in the fifth and sixth decades, presenting as a diffuse but symmetrical, papular or annular eruption of more than ten skin lesions, and often hundreds.
Granuloma multiforme is a cutaneous condition most commonly seen in central Africa, and rarely elsewhere, characterized by skin lesions that are on the upper trunk and arms in sun-exposed areas. It may be confused with tuberculoid leprosy, with which it has clinical similarities. The condition was first noted by Gosset in the 1940s, but it was not until 1964 that Leiker coined the term to describe "a disease resembling leprosy" in his study in Nigeria.
Porokeratotic eccrine ostial and dermal duct nevus (PEODDN) is a skin lesion that resembles a comedonal nevus, but it occurs on the palms and soles where pilosebaceous follicles are normally absent. It is probably transmitted by paradominant transmission.
Palmoplantar keratoderma with deafness, also known as Palmoplantar keratoderma-deafness syndrome is a rare genetic disorder which is characterized by either focal or diffuse early-onset palmoplantar keratoderma and sensorineural deafness. Transmission is autosomal dominant with incomplete penetrance.
