Erythrokeratoderma: causes, symptoms, diagnosis, treatment

Erythrokeratoderma occupy an intermediate position between diffuse and localized forms of keratoses. There are several forms of spectrocardoderma: the figurative variable Mendes da Costa; congenital progressive symmetric Gottron; ichthyosis linear envelope of Comet; genodermatosis kokardovidny Degosa, etc., the relationship between which is still unclear. It is possible that they are variants of one disease.

Erythrokeratoderma figurative variable Mendes da Costa (syn. Keratosis variabilis figurata) - a frequent disease from the group of spectrocokerolermia, is inherited by autosomal dominant type. The localization of the gene is 1p36.2-p34. It appears, as a rule, in the first year of life, erythematous-squamous eruptions of curly, bizarre outlines that change borders within a few hours or days. In the long-term foci of erythema, a slight, more pronounced peripheral hyperkeratotic changes, which vary less than erythema. This disease also describes diffuse universal hyperkeratosis, palmar-plantar keratoderma, achromatic spots, changes in nail plates, vesicular rashes.

Pathomorphology. Acanthosis, papillomatosis, pronounced lamellar hyperkeratosis, horny plugs in the mouths of hair follicles. A granular layer of normal thickness. Sometimes small pervasive inflammatory infiltrates are observed in the papillary layer of the dermis. Some authors observed parakeratosis, spongiosis and eosinophilic homogeneous structures with the remains of nuclei in the stratum corneum, 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; presumably, there is retention hyperkeratosis.

Congenital symmetric progressive Gritron erythrokeratodermia is inherited, probably, by autosomal dominant type. Usually in childhood appear symmetrically located eruptions in the form of erythema red-brown color with lamellar ecdysis, mainly in its marginal zone, surrounded often by the corona of hyperpigmentation. Characteristic is the damage to the skin around the mouth and in the nasolabial folds, peeling and erythema of the scalp, large plaques on the elbows and knees, stripy hyperkeratosis on the flexor surfaces of the joints. The foci grow slowly in size. There are cases of hyperkeratosis of the palms and soles, combination with cataracts.

Pathomorphology. Acanthosis with uneven expansion and elongation of epidermal processes, hyperkeratosis, focal parakeratosis near hair follicles such as cornoid plate, follicular hyperkeratosis is detected. The granular layer is slightly thickened, and vacuole dystrophy of individual epithelial cells is noted. In the upper part of the dermis are moderate perivascular lymphohistiocytic infiltrates. At electron microscopy in horny scales there are lipid drops, desmosomes. In the cells of the spine-like layer, a thickening of the tonofilament is found and an increase in their number. In the basal layer, the amount of desmosomes is increased. Histogenesis is not clear, it is suggested that the excessive formation of desmosomes and the pathology of tonofilamentes play a role in the development of the pathological process.

Ichthyosis, the linear envelope of Comet (syn. Dyskeratosis ichthyosiformis congenita migrans) is a rare disease that is inherited, probably, by an autosomal recessive type. The clinical feature of serpiginous-polycyclic erythematoma-squamous migratory foci is a double scaly edge. Characteristic is the damage to the folds of the skin. The majority of patients have bamboo-like hair, as with the syndrome of Netergone.

Pathomorphology. The changes are nonspecific, they show hyper- and parakeratosis. Moderate acanthosis, intra- and intercellular edema, especially pronounced in the peripheral active zone of lesions, sometimes with the formation of vesicles. In the dermis are the dilated vessels of the papillary layer and small perivascular infiltrates consisting of lymphocytes and histiocytes.

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