Dyskeratosis Follicular

Dyskeratosis Follicular is a rare genetically determined skin disease characterized by dysplasia of the epithelial elements of the follicle without the presence of malignant neoplasms.

The disease is typical for elderly and senile people; it is often registered among relatives of the proband (the patient’s mother) 3-5 times more often than among healthy women.

During the first examination, the presence of the disease is confirmed in approximately one third of patients, and as experience in pathoanatomical practice accumulates, it is detected more and more often. An increase in incidence was noted in areas of Siberia and the Far East, which may be due to the influence of cold factors.

The difficulty of diagnosing the disease is also due to the lack of a unified research method and the insufficient development of classification methods. In this regard, the classification of various authors included various clinical variants of the disease, having both similar and sometimes contradictory diagnostic criteria. Skin damage begins with the follicles and then spreads to the epidermis, atrophy, hyper- and hypopigmentation are possible. Older people over 60 years of age are more prone to pigmentation. Pigmented areas often have irregular contours and reach large sizes. Pigmentation color can vary from yellow to dark brown. The mouths of the sebaceous glands are also affected. Often the disease is accompanied by pathological hair growth (hypertrichosis). Small erosions (atopic excoriation) and ulcerations may occur, which are prone to prolonged healing, scarring, and deformation of the fingertips. X-ray examination usually reveals only nodular osteoporosis. The fibrotic stage, which requires the use of painkillers, develops less frequently: the number of patients with the fibrotic stage increases with age, amounting to approximately 3-8%.

Risk factors for the development of dyskeratosis include sunlight and exposure to cold. Some jobs such as painting houses and spraying chemicals and solvents in factories increase the risk of this disease. In typical cases, follicular hyperplasia and dyskeratoma of the eyelid develop, manifested by impaired growth of eyelashes, their partial loss, unequal size, and thickening of the barrel. There are no diagnostic criteria for follicular dyskirotic melanocytic hyperplasia of the eyelids, and the diagnosis is made based on the clinic and pathomorphological examination of skin or eyelash biopsies. This form has a pronounced predisposition to malignancy, which is manifested by a number of symptoms (keratotic sebaceous papules, ulcerations and crusts) and pigmented or pyogenic plaques. The hair follicles are dilated in a groove-like manner, and flattened grayish-yellow papules covered with scales are visible in the epidermis from the ear surface. Papules are often accompanied by hirsutism, sometimes multiple. The boundaries of the skin pattern are deformed. Ulcers sometimes form under the affected areas. The danger of malignancy that develops in this case is characterized by a change in the color of the epithelium (previously ivory) with brown pigmentation and an inflammatory reaction.