Palm and plantar keratoderma: causes, symptoms, diagnosis, treatment

Palmoplantar keratodermas are a large group of diseases that are very different in their morphology. Some of them are independent diseases, others are part of numerous syndromes, and others are one of the manifestations of diffuse keratoses. Histogenetically, the entire diversity of clinical manifestations can be reduced to several histomorphological types.

All palmoplantar keratodermas have common histological features: varying degrees of acanthosis, hyperkeratosis, sometimes focal parakeratosis; changes in the basal layer of the epidermis and basement membrane are absent. As a rule, there is no inflammatory reaction in the dermis, only sometimes small perivascular infiltrates are found in its upper part. The features that allow dividing palmoplantar keratodermas into different types include changes in the structure of the granular and spinous layers of the epidermis: hyperkeratosis with an increase in the number of layers of the granular layer (granulosis), epidermolytic hyperkeratosis. atrophy or absence of the granular layer. Hyperkeratosis and granulosis are observed in the overwhelming majority of palmoplantar keratodermas, both in diffuse and limited forms.

The following nosological entities are classified as diffuse forms of keratoderma.

Tosta-Unna palmoplantar keratoderma

Inherited in an autosomal dominant manner, characterized by diffuse lesions of the palms and soles. Changes in the area of the interphalangeal joints of the hands have also been described. It exists from birth or develops during the first year of life, rarely - at a later age. There is diffuse keratosis of the palms and soles with a strip of congestive vision along its edge. Painful cracks are common.

Pathomorphology. Pronounced hyperkeratosis, granulosis, hypertrophy of sweat glands, sometimes a picture of epidermolytic hyperkeratosis, but in such cases it is necessary to exclude a limited form of bullous ichthyosiform erythroderma. Electron microscopic examination revealed atypical keratohyaline granules of two types - less electron-dense granular structure and more electron-dense, attached to the former.

Werner's palmoplantar keratoderma

It is inherited in an autosomal dominant manner. Mutation of the gene encoding keratin 9, located in the locus 17ql2-q21, has been identified. The disease develops in the first weeks of life. The clinical picture is similar to Tosta-Uina palmoplantar keratoderma. Hyperhidrosis and thickening of the nail plates are noted. Spontaneous exfoliation of the horny masses, occurring 1-2 times a year, has been described.

Pathomorphology. Similar to that of congenital bullous ichthyosiform erythroderma, which is confirmed by electron microscopy. It can be assumed that the histogenesis of the disease is based on disturbances in the formation of tonofibrils. Biochemical analysis revealed the appearance of low-molecular keratins in the epidermis, indicating a disturbance in the differentiation of epithelial cells.

Keratoderma mutilans

Inherited in an autosomal dominant manner, characterized by the presence of keratosis with a honeycomb surface on the palms and soles, keratotic lesions of stellate outlines on the back of the hands and feet, the inner surface of the wrist joints, and ring-shaped bands of the fingers (pseudo-aingum). Onychodystrophies are common, and diffuse alopecia has been described.

Honeycomb keratosis, but without constriction, is also observed in palmoplantar keratoderma associated with deafness, in which, as in mutilating palmoplantar keratoderma, there are keratotic foci on the dorsum of the hands and feet with a transition to the inner surface of the wrist joints.

Pathomorphology: hyperkeratosis with hypergranulosis.

Diffuse palmoplantar keratoderma

With an autosomal dominant type of inheritance (gene docus - 17q23-ater) can be combined with esophageal cancer (Howel-Evans syndrome), Keratosis usually develops at 5-15 years, esophageal cancer - after 30 years. Multiple basaliomas can be observed simultaneously.

Meleda Island Keratoderma

Syn. Melela Island disease is inherited in an autosomal repressive manner. Clinically, it is characterized by diffuse keratosis of the palms and soles, a pronounced inflammatory reaction in the form of an erythematous halo around the keratotic lesions with lesions extending to the dorsum of the hands and feet, the knee and elbow joints, the lower third of the forearms and shins (in the form of "gloves and socks"). Contractures and adhesions of the fingers are common. A combination with pseudoaingum has been described. The disease is accompanied by hyperhidrosis and changes in the nail plates, leukokeratosis is also possible.

Pathomorphology. Electron microscopy reveals keratohyalin granules of complex structure, consisting of a less dense granular core and a denser peripheral zone associated with tonofilaments. Such granules are most often located in epithelial cells located in the area of the openings of sweat glands.

Keratoderma described by A. Greither (1952) is similar in clinical manifestations to Meleda Island disease. However, this form is inherited in an autosomal dominant manner, is characterized by less pronounced hyperkeratosis, the presence of changes in other areas of the skin similar to those observed in erythrokeratoderma, a less severe course, and improvement with age.

Papillon-Lefevre keratoderma

Syn. Papillon-Lefevre syndrome is inherited in an autosomal recessive manner. The clinical picture is similar to keratoderma of the island of Meleda. Skin lesions are combined with periodontal disease, inflammation of the gums and papillae of the tongue, and susceptibility to various infectious diseases. Sometimes growth retardation, hypotrichosis, calcification of the meninges, and a combination with congenital bronchiectasis are observed.

Pathomorphology: massive compact hyperkeratosis and hypergranulosis; in erythematous-squamous lesions in the area of large joints and the dorsal surface of the hands and feet, the histological picture resembles pityriasis versicolor pilaris (Devergie's disease): hyperkeratosis with alternating areas of ortho- and parakeratosis, uneven acanthosis, minor perivascular inflammatory infiltration in the papillary layer of the dermis.

Olmstead syndrome

It is a combination of diffuse palmoplantar keratoderma with clear edges, onychodystrophy, constriction of the fingers and periorificial keratosis. In addition to the listed signs, universal alopecia, leukokeratosis, and dental anomalies are described.

Limited palmoplantar keratoses

A collective term used for all limited (focal, linear) forms of keratoderma. The inheritance pattern is autosomal dominant. Clinical manifestations of the disease may appear in adolescence or in adults. In large-focal forms of keratoderma, coin-shaped rounded keratotic lesions are found on the palms and soles, most pronounced at the sites of pressure, and large, isolated or combined with linear keratoses lesions in the area of the flexor surfaces of the fingers. A combination with spiral curly hair may be observed. Electron microscopic examination in one case revealed edema of epithelial cells, increased density of tonofilaments in the suprabasal region, vacuolization of spinous cells, changes in the structure of keratohyaline granules and lipid droplets in the stratum corneum.

Papular palmoplantar keratodermas are characterized by a diffuse nature and smaller sizes of keratotic foci. They develop in the first years of life (Brauer's keratoderma) or at the age of 15-30 (Buschke-Fischer keratoderma). Clinically, they are characterized by multiple flat, hemispherical or warty keratinized foci, round or oval in shape, usually located in isolation over the entire surface of the palms and soles, and not only in places of pressure. After removal of the horny masses, a crater- or saucer-shaped depression remains. A. Greither (1978) considers the listed forms of papular keratoderma to be identical.

Punctate congenital acrokeratoderma

Syn. punctate keratosis of the palms and soles is characterized by the appearance on the palms and back of the hands of small keratotic papules the color of normal skin with a smooth shiny surface. Histologically, FC Brown (1971) identified parakeratotic columns similar to those observed in porokeratosis of Mibelli. DG Robestria et al. (1980) found intranuclear disorders in the form of multiple hypertrophied nucleoli in the cells of the basal and spinous layers using electron microscopy, which, according to the authors, contribute to the development of hyperkeratosis. A combination of this disease with cancer of the internal organs has been described. MJ Costello and RC Gibbs (1967) consider papular and punctate keratodermas to be synonyms.

Keratoderma with translucent papules is probably a variant of punctate congenital acrokeratoderma. It is also inherited in an autosomal dominant manner and is characterized by yellowish-white, translucent papules with a smooth surface, sometimes with punctate depressions in the center, merging into plaques. It is combined with thin scalp hair and atopy.

Punctate keratosis of the palmar lines is characterized by the presence of small hyperkeratotic plugs on the palms and soles, located in the depressions of the skin lines, which are painful when pressed.

Palmoplantar keratoderma with twisted hair is an autosomal dominantly inherited disease characterized by the presence of rounded keratosis lesions on the palms and soles. Pathological changes in hair were confirmed by scanning electron microscopy. Cysteine deficiency was histochemically detected in the hair.

Rhnner-Hanhart syndrome

Syn.: cutaneous and ocular tyrosinosis, tyrosinemia type II is characterized by painful palmar-plantar keratotic lesions, herpetiform corneal dystrophy and mental retardation. Without treatment, diffuse keratoderma develops with age, there may be blisters. The type of inheritance is autosomal recessive, the gene locus 16q22.1-q22 is affected. Histologically, in addition to the signs common to this group of keratodermas, eosinophilic inclusions are detected in the cells of the spinous layer. Electron microscopic examination reveals an increase in the number of tonofilaments in spinous epithelial cells, tubular channels in the bundles of tonofilaments. Histogenesis is based on a deficiency of the enzyme tyrosine aminotransferase, which leads to the accumulation of tyrosine in the blood and tissues. It is assumed that L-tyrosine molecules can promote the formation of additional cross-links. This leads to the thickening of tonofibrils in epithelial cells.

Palmoplantar nummular keratoderma

The so-called painful calluses are inherited in an autosomal dominant manner. It develops in childhood or adolescence and is characterized by the presence of limited large hyperkeratotic lesions localized in pressure areas: on the soles, at the base and on the lateral surfaces of the toes, on the fingertips, painful when pressed. Blisters along the edges of the lesions, subungual or periungual hyperkeratosis, thickening of the nail plates, and hyperkeratotic lesions on the shins are described. Epidermolytic hyperkeratosis is observed histologically.

Acrokeratoelastoylosis Costa

It develops in childhood. Clinically it is manifested by small, sometimes merging papules of a scaly consistency, grayish in color, translucent, with a shiny surface, located on the palms and soles, along the edges of the fingers, in the area of the Achilles tendon. Histochemically in the dermis in the lesions thickening and fragmentation of plastic fibers are revealed, electron microscopically - changes in their amorphous part, disruption of the arrangement of microfibrils. There are no changes in the granular layer.

It should be noted that a large group of palmoplantar keratodermas has not yet been classified either clinically or histologically. The literature contains morphological descriptions of only individual cases. In this regard, diagnostics, especially differential, of these diseases presents great difficulties.

Differences in the clinical characteristics of rashes and the type of inheritance, the features of the course of diseases within the groups we have identified allow us to assume their different pathogenesis with a similar histological picture.

[ 1 ], [ 2 ], [ 3 ], [ 4 ]