HABER SYNDROME

From Big Medical Encyclopedia

HABER SYNDROME (N of Haber, the modern English dermatologist) — the hereditary symptom complex including a combination of rozatseapodobny rash to intraepidermalny epitheliomas and hypersensitivity of skin to sunshine.

The first case was revealed and histologically studied by Haber in 1959. G.'s description of page belongs to Sanderson and Wilson (To. V. Sanderson, H. T. Wilson, 1965). Of page is inherited autosomal is prepotent.

Patogistologiya

In epidermis of face skin — an acanthosis, a parakeratosis, sites of intercellular hypostasis, strengthening of mitotic activity of cells of a basal layer; in a derma — permanent expansion and increase in number of vessels of superficial circulatory network, proliferation of undifferentiated cells of sebaceous glands (apparently, as a result of a reactive hyperplasia), sites of fibrosis. In the centers of damage of skin of a trunk gistol, changes (cellular atypia, a dyskeratosis, multiple mitoses) correspond to an intraepidermalny epithelioma.

The clinical picture

the Disease is shown at children's age by the resistant erythema in a nose, cheeks, a forehead and a chin which is amplifying after stay in the sun and followed by a slight itch. Against the background of an erythema appear rozatseapodobny rash — small follicular dense papules of red color, sites of a peeling, the pressed small scars, teleangiectasias. On the closed sites of skin, hl. obr. on a back, a breast, the centers of a hyperkeratosis and warty formations of the small sizes gradually form: subjective feelings are absent. In cheeks and a chin — an easy hypertrichosis.

The diagnosis

the Diagnosis is established on the basis of clinical data in combination with gistol, a research (detection of intraepidermalny epitheliomas). To differentiate Of page follows with acne rosacea (see. Eels ), at to-rykh formation of intraepidermalny epitheliomas is not observed and to-rye are shown at more advanced age and are often connected with dysfunction went. - kish. path. From a photodermatitis (see. Photodermatoses ) Of page differs in absence gistol, signs of a solar keratosis and elastic dystrophy of connecting fabric, and also existence of intraepidermalny epitheliomas on the closed sites of an integument.

Treatment

Treatment: corticosteroid ointments, rentgeno-and radiotheraphy of tumors.

The forecast

the Course of a disease chronic with aggravations in spring and summer time. The malignancy of separate educations is possible.

Prevention aggravations — photoprotective creams («Beam», «Board», etc.).


Bibliography: Sanderson K. V. and. Wilson H. T. Haber’s syndrome, Brit. J. Derm., v. 77, p. 1, 1965; about N and e, Haber syndrom, Derm. Wschr., Bd 153, S. 617, 1967; Seiji M. O t a k i N. Haber's syndrome, Arch. Derm., v. 103, p. 452, 1971.

S. S. Kryazheva.

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