Red papillary hair lichen: causes, symptoms, diagnosis, treatment

Pityriasis rubra pilaris (synonyms: Devergie's disease, acuminate red lichen) is a heterogeneous disease consisting of both hereditary forms transmitted autosomal dominantly, and sporadic, acquired forms clinically close to follicular psoriasis. Of the five clinical types of the disease identified by WAD Griffiths (1980), the atypical juvenile is hereditary. The acquired and hereditary forms of the disease are clinically and histologically similar. Clinical manifestations of the hereditary type of the disease occur in childhood, most often with lesions of the palms, where reddish-yellow erythema with scaling appears. Keratosis of the palms and soles develops. Then follicular papules with perifollicular erythema appear, which, merging, form large foci. In adults, erythroderma with islands of unchanged skin often develops. The mucous membranes of the oral cavity and eyes may be affected, with the development of conjunctivitis, corneal dystrophy, and ectropion. The appearance of horny cones on the back of the fingers (Besnier's symptom) and changes in the nail plates are characteristic. Allergic dermatitis, especially of the palms and soles, may contribute to the development of the disease.

The causes and pathogenesis of red pityriasis versicolor hairy lichen are unknown, in some cases a hereditary predisposition is noted. In recent years, an opinion has been expressed about two types of red pityriasis versicolor hairy lichen, one of which begins soon after birth, in childhood or adolescence (childhood type), and the other occurs in adulthood (adult type). It is believed that the childhood type of the disease is hereditary, and the adult type is acquired.

Of the existing theories of the pathogenesis of red pityriasis versicolor, the most recognized is the concept of deficiency or insufficient absorption of vitamin A, a decrease in the level of retinol-binding protein. In addition, factors such as endocrine disorders, nervous disorders, intoxication, etc. play a role in the development of red pityriasis versicolor.

Symptoms of Pityriasis Versicolor Pilaris

At the onset of the disease, isolated follicular pointed nodules appear, pink-red, bright red or dark red, bran-like scaly, with a small horny spine in the center. Later, as the papules increase in size or merge, plaques of yellowish-red color with an orange tint are formed, which are more or less infiltrated, covered with whitish scales and dotted with sharply expressed skin grooves (lichenification). Stroking the affected areas gives a feeling of grater. The favorite localization of the rash is the extensor surfaces of the extremities, especially the dorsum of the fingers, where pathognomonic changes for this dermatosis appear in the form of follicular horny cones described by Besnier, although the rash can also occur on other areas of the skin. Another pathognomonic symptom is islets of healthy skin of irregular outlines with horny spines located on them, striking against the general background of yellowish-red infiltrated skin. The rash is usually symmetrical. On the scalp there is a large layer of tightly seated dry bran-like scales (asbestos scales). The skin of the face is pink-red, with flour-like peeling. On the palms and soles there is focal or diffuse keratoderma - the skin is hyperemic, thickened, covered with scales and cracks. Universal damage to the skin of the erythroderma type may develop. Damage to the nail plates on the hands and feet is one of the characteristic signs of dermatosis. In this case, longitudinal or transverse striation, clouding of the nail plates and pronounced hyperkeratosis are noted.

The disease usually begins in childhood or adolescence (childhood type), but cases of later onset (adult type) are relatively common. Patients usually experience slight itching and complain of tightness of the skin. Sometimes, the course of pityriasis versicolor is very similar to psoriasis, and then they talk about a psoriasis-like form, or a psoriasis-like variant of the course of pityriasis versicolor versicolor.

Histopathology. Hyperkeratosis with follicular plugs, slight parakeratosis and granulosis, vacuolar degeneration of the basal layer cells are noted. In the upper dermis, a perivascular infiltrate is observed, consisting mainly of polymorphonuclear leukocytes and lymphocytes located around the vessels and near the hair.

Pathomorphology. Uneven acanthosis, hyperkeratosis with foci of parakeratosis, horny plugs in the mouths of hair follicles and epidermal depressions, on the sides of which parakeratosis is often expressed, are noted. The granular layer is expanded, of uneven thickness, consists of 1-4 rows of cells. Granular epithelial cells are often vacuolated. In the upper part of the dermis there is edema, vasodilation, perivascular infiltrates. M. Larregue et al. (1983) note that follicular keratosis and perivacular infiltrates are not always expressed. Histochemical examination revealed an increase in the activity of hydrolytic enzymes and a positive reaction to phospholipids in the oral layer. Electron microscopic examination revealed a moderate increase in the activity of azilic epithelial cells, widening of intercellular spaces, and a decrease in the number of tonofilaments and desmosomes. The granular layer is widened, according to L. Kanerva et al. (1983), and has up to 9 rows. Keratohyalin granules are mostly unchanged, but there are areas of their disaggregation. The number of lamellar granules is increased, especially in the intercellular spaces. Between the granular and horny layers there are 1-2 rows of narakeratotic cells - a transition zone. According to O. Braun-Falco et al. (1983), it consists of 3 rows. The vessels of the microcirculatory bed are distinguished by increased activity of endotheliocytes and pericytes containing a large number of organelles. The basal layer consists mainly of an amorphous substance. Deposits of the same substance are also noted under the basal membrane of the epidermis, which may be associated with exudative processes. The horny scales contain many lipid droplets, which distinguishes this disease from other keratoses.

Histogenesis. In addition to diffuse parakeratosis, follicular hyperkeratosis is expressed. Many enzymes participate in the process of keratinization, which occurs in the epidermis and hair follicles. At the same time, the formation of trichohyalin, which is qualitatively different from the keratin of the epidermis, in the hair follicles also requires the participation of enzymes specific to this type of keratinization. In Devergie's disease, there is probably an enzymatic defect common to both types of keratinization. It is assumed that an important role in the pathogenesis of Devergie's disease is played by vitamin A deficiency or a violation of its metabolism, in particular a defect in the synthesis of retinol-binding protein. It has been established that the concentration of this protein in the blood of patients is close to normal.

In differential diagnostics of Devergie's disease and non-bullous ichthyosiform erythroderma, clinical features and the type of inheritance are of great importance. The later onset of Devergie's disease, the characteristic color of erythema, islands of unchanged skin on its background, and pronounced follicular keratosis usually help in diagnostics. Due to the vagueness of the histological picture in these two diseases, electron microscopic and biochemical examination of the skin is necessary, especially for n-alkanes. It is more difficult to differentiate Devergie's disease from psoriatic erythroderma. However, pronounced follicular hyperkeratosis and granulosis in Devergie's disease, massive hyperkeratosis and parakeratosis with more pronounced acanthosis in psoriasis may be distinctive features of these two diseases.

Treatment of red pityriasis versicolor hair

Vitamin A is used in high doses (300,000-400,000 mg per day), neogigazone (0.5-1 mg/kg of patient weight), PUVA and Re-PUVA therapy, methotrexate, glucocorticosteroids. Externally - keratolytic agents and local corticosteroid drugs.

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