Kirle's disease belongs to the group of keratoses (see the complete body of knowledge); first described by J. Kirle in 1916. The etiology is unknown. The genetic nature of dermatosis is assumed, great importance is also attached to the violation of vitamin A metabolism, disorders of carbohydrate metabolism. Patohistology. A pronounced hyperkeratosis is characteristic with the formation of horny cones that penetrate into the epidermis, both in the area of propecia pills follicles and outside them. In some areas, horny masses violate the integrity of the epidermis and penetrate into the skin itself, which causes the formation of granulomas consisting of lymphocytes, fibroblasts, epithelioid cells and a significant number of giant cells of foreign bodies; the epidermis around the circumference of the horny cone in a state of acanthosis (Figure 1). In the subcutaneous adipose tissue - cell infiltration, the appearance of acid mucopolysaccharides, a decrease in the number of elastic fibers.
The primary element is a follicular, less often parafollicular horny nodule the size of finasteride Hair loss, yellowish in color; as the nodules increase, they gradually acquire a brown-red color and a warty appearance due to horny layers. When the nodules merge, plaques of various sizes are formed, which mostly have polycyclic outlines (Figure 2). After removal of the horny layers, sometimes consisting not only of scales, but also kokorok scales, funnel-shaped depressions are found under them, slightly weeping, less often bleeding. Healing occurs with superficial scars, pigmentation temporarily remains. The lesion is usually localized on the extensor surface of the extremities, but sometimes has a disseminated character involving the scalp, face, trunk, and very rarely the oral mucosa. The course is chronic, sometimes with relapses. The general condition of the patients is not disturbed, there is no itching.
Micropreparation of the skin in Kirl's disease: hyperkeratosis with the formation of horny cones (I), acanthosis (2), forming granuloma, consisting of lymphocytes, fibroblasts, epithelial cells (3). clinical picture. Kirle disease develops at any age, but more often in boys 10-12 years old.
The diagnosis is not always easy to establish. The disease differs from warty lichen planus Kirle by the absence of itching and primary rashes typical of lichen planus (see Lichen red flat). The disease differs from red hairy lichen Kirle in the absence of propecia pills Besnier cones on the back of the phalanges of the fingers, the presence of polycyclic foci, and the possibility of easy removal of horny layers. According to the clinical picture of Kirle, the disease may resemble Darier's disease (see Darier's disease), differing from it by the presence of funnel-shaped depressions after the removal of horny masses. In doubtful cases, they resort to a histological examination: the phenomena of dyskeratosis for Kirle are not characteristic of the disease. It should be noted that in exceptionally rare cases, the penetration of horny masses into the skin proper (Kirle phenomenon) also occurs in some other diseases.
Follicular horny nodules on the skin, sometimes merging into plaques in Kirl's disease. Treatment: long-term use of vitamin A, keratolytic ointments, ointments with retinoic acid (vitamin A), subsequent UV irradiation; individual foci can be removed surgically, by methods of diathermocoagulation, cryotherapy (liquid nitrogen). Presumably the type of inheritance is autosomal recessive, since first-degree relatives sometimes suffer from Kirle disease. The age of onset is 20-70 years, on average 30-40 years. Children get sick very rarely. Women get sick more often than men.
The onset of the disease is gradual. It is characterized by a rash of scattered dense papules with keratinization in the center in the form of a horny plug, follicular, less often parafollicular, sometimes painful when pressed.there are papules that do not differ in color from the surrounding skin, 2-3 mm in diameter, then they gradually increase, reaching a size of 8-10 mm. The color of the rashes around the keratinized center is grayish, brownish-red or yellowish, without inflammatory changes along the periphery of the elements. Horny masses can give the surface of the papules a warty appearance.
The pathogenesis is unknown. The development of the disease during treatment with penicillamine is described. Patients often have diabetes mellitus and chronic renal failure, less often liver failure, heart failure, hypothyroidism, hyperlipoproteinemia. Currently, they are classified as acquired perforative dermatosis.
The rash is prone to peripheral growth and fusion with the formation of dry warty plaques of polycyclic outlines. Ichthyosiform skin changes are possible. After removal of the corneal plug, a slightly moist or bleeding crater-like depression is found (Kirle phenomenon). Elements are located mainly on the extensor surface of the legs and forearms, buttocks, sometimes on the face, trunk, scalp, much less often on the palms, soles. Mucous membranes are usually not affected. Fresh rashes are accompanied by slight itching or do not bother the patient at all.
Older, larger lesions are painful, especially when pressed. In places of scratching, a linear arrangement is often observed (positive Koebner phenomenon). Infectious complications are possible with skin injuries. The disease is chronic. New rashes appear as the old ones disappear. The disease often recurs in the same place. regression remains an atrophic, unevenly hyperpigmented scar.
The diagnosis is established on the basis of a characteristic clinical picture and histological examination data. Histological examination reveals extrafollicular intussusceptions of the thinned epidermis filled with horny plugs with areas of basophilia. Follicular hyperkeratosis is also pronounced, sometimes layered, with focal parakeratosis. Violation of the integrity of the epidermis in some areas is accompanied by an inflammatory reaction. It is recommended to examine patients to exclude latent diabetes with chronic renal failure.
It is clinically manifested by a rash on the extensor surfaces of the arms and legs of small dirty-red papules covered with horny scales, capable of dissemination. Removal of horny scales opens weeping surfaces with subsequent outcome in a shallow pigmented scar. The diagnosis is made on the basis of anamnesis, clinical picture, the presence of concomitant pathology, clinical and laboratory examination. Morphology is nonspecific. Treatment is aimed at radical removal of foci of hyperkeratosis.
0.1% phenol with 10% glycerol Doxycycline Surgical debridement Transcutaneous electrical nerve stimulation Topical corticosteroids Keratolytics (eg, salicylic acid and urea) Kirle's disease is a dermatosis of unclear etiology, characterized by a relapsing course with the formation of perifollicular foci of hyperkeratosis in the dermis. Causes of Kirle's disease Symptoms of Kirle's disease Diagnosis of Kirle's disease Treatment of Kirle's disease Prices for treatment.
The Austrian dermatologist Josef Kirle first described the clinical manifestations of the disease in 1916. The merit of the author, whose name has since been borne by hyperkeratosis, is that he singled out this pathology as an independent nosological unit, while before him penetrating hyperkeratosis was considered only as a symptom of many skin diseases. Interestingly, the nature of his work contributed to this. J. Kirle was engaged in histopathology at the Vienna Skin Clinic, the main work of his life was a two-volume book on skin histobiology. Based on the comparative morphological characteristics of many keratoses, the scientist managed to prove the uniqueness of this pathology (the penetrating nature of hyperkeratosis). The relevance of the disease is determined by the presence of systemic disorders that can lead to death.
The etiology of the disease is unknown. Kirle's disease can occur spontaneously as a result of metabolic disorders (synthesis of vitamin A, lipids), have a genetic component (family history of the disease). In the vast majority of cases, Kirle's disease is associated with systemic disorders (chronic renal and cardiovascular failure, diabetes mellitus, liver cirrhosis).
The primary element is a brown papule (follicular nodule) the size of a millet, yellowish-silver in the center (keratin). The rash is localized more often on the extremities, in the elbow and popliteal folds. Papules transform into plaques, which, merging with each other, form efflorescences (local foci of rashes) with foci of necrosis or hyperkeratosis in the center. Dermatosis tends to generalize the process (with the exception of the palmsand soles). When removing the horny layers, erosion is exposed in the form of a shallow funnel, weeping, bleeding slightly, leaving behind a pigmented superficial scar. The outcome of independent involution of elements is atrophic scarring.
There are no subjective sensations associated with skin manifestations. In rare cases, the patient is worried about itching. The objective condition depends on the presence of concomitant pathology. Some practicing dermatologists believe that the occurrence of penetrating keratosis is the main symptom of an impending systemic disease. Dermatosis has a chronic course, relapses are not excluded. There are no age and gender components. Diagnosis of Kirle's disease.
The main attention in the diagnosis of dermatosis is paid to the presence of concomitant systemic pathology. They conduct a consultation of doctors consisting of a dermatologist, endocrinologist, nephrologist, neurologist, gastroenterologist, cardiologist; complete clinical and laboratory examination (OAC, OAM, biochemistry, special stress tests in order to establish the safety of the functions of the organ).
Solitary, limited foci of rashes are removed radically: small papules - with a laser, radio wave therapy, large ones - surgically. A consultation with a dermatocosmetologist is indicated for the purpose of electrocoagulation or cryodestruction of foci of local keratosis, phototherapy. Conservative therapy includes correction of carbohydrate and lipid metabolism, kidney pathology, diseases of the cardiovascular system, and other concomitant pathologies. Background therapy involves long-term courses of vitamin A, E in capsules, with resistance to the process, the appointment of glucocorticoids (betamethasone) is indicated. Externally, keratolytic ointments, UVI, bran baths, mud therapy are used. The prognosis in terms of Generic Propecia pathology is favorable. Somatic pathology requires constant attention.
Localization of Kirle's disease Features of Kirle's disease on the face Causes of Kirle's disease Symptoms and manifestations of Finasteride disease Treatment of Kirle's disease Treatment with folk remedies Approaches to the prevention and prognosis of Kirle's disease Photo of Kirle's disease.