Erythrokeratoderma: causes, symptoms, diagnosis, treatment

Erythrokeratoderma occupies an intermediate position between diffuse and localized forms of keratoses. There are several forms of erythrokeratoderma: figured variable of Mendes da Costa; congenital progressive symmetrical of Gottron; linear ichthyosis circumflex of Komel; genodermatosis cockadei of Degos, etc., the relationship between which is still unclear. It is possible that they are variants of the same disease.

Erythrokeratoderma figurata variable Mendes da Costa (syn. keratosis variabilis figurata) is a common disease from the group of erythrokeratodermas, inherited in an autosomal dominant pattern. Localization of the gene is 1p36.2-p34. It usually manifests itself in the 1st year of life with erythematosquamous rashes of figured, bizarre outlines, changing boundaries over several hours or days. In long-existing foci, erythema is insignificant, more pronounced at the periphery of hyperkeratotic changes, which vary less than erythematous ones. Diffuse universal hyperkeratosis, palmar-plantar keratoderma, achromatic spots, changes in the nail plates, vesicular rashes have also been described in this disease.

Pathomorphology. Acanthosis, papillomatosis, significantly expressed lamellar hyperkeratosis, horny plugs in the mouths of hair follicles. Granular layer of normal thickness. Sometimes small perivascular inflammatory infiltrates are observed in the papillary layer of the dermis. Some authors observed parakeratosis, spongiosis and eosinophilic homogeneous structures with nuclear remnants in the horny layer, as well as a significant decrease in the number of intraepidermal macrophages.

The histogenesis of the disease is unclear. Incubation of sections with 3H-thymidine reveals normal cell proliferation; retention hyperkeratosis appears to be present.

Congenital symmetrical progressive erythrokeratoderma Gottron is inherited, probably by an autosomal dominant type. Usually in childhood, symmetrically located rashes appear in the form of red-brown erythema with lamellar peeling, mainly in its marginal zone, often surrounded by a rim of hyperpigmentation. Skin lesions of the face around the mouth and in the nasolabial folds, peeling and erythema of the scalp, large plaques on the elbows and knees, stripe hyperkeratosis on the flexor surfaces of the joints are characteristic. The lesions slowly increase in size. Cases of hyperkeratosis of the palms and soles, a combination with cataracts are observed.

Pathomorphology. Acanthosis with uneven expansion and elongation of epidermal outgrowths, hyperkeratosis, focal parakeratosis near hair follicles of the cornoid plate type, follicular hyperkeratosis are detected. The granular layer is slightly thickened, vacuolar degeneration of individual epithelial cells is noted. In the upper part of the dermis, there are moderate perivascular lymphohistiocytic infiltrates. Electron microscopy reveals lipid droplets and desmosomes in the horny scales. Thickening of tonofilaments and an increase in their number are found in the cells of the spinous layer. The number of desmosomes is increased in the basal layer. Histogenesis is unclear, it is assumed that excessive formation of desmosomes and pathology of tonofilaments play a role in the development of the pathological process.

Ichthyosis linearis circumflexa Komel (syn. dyskeratosis ichthyosiformis congenita migrans) is a rare disease, inherited, probably, by an autosomal recessive type. The clinical feature of serpiginous-polycyclic erythematosquamous migratory lesions is a double scaly edge. Skin folds are affected characteristically. Most patients have bamboo-like hair, as in Nethergon syndrome.

Pathomorphology. The changes are non-specific, revealing hyper- and parakeratosis, moderate acanthosis, intra- and intercellular edema, especially pronounced in the peripheral active zone of the lesions, sometimes with the formation of bubbles. In the dermis - dilated vessels of the papillary layer and small perivascular infiltrates consisting of lymphocytes and histiocytes.

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