May
19
23:01
Genetic diseases

Hailey - Hailey disease

Hailey - Hailey disease

Hailey-Hailey (aka benign familial pemphigus) - a rare genetic dermatosis.Clinical manifestations of the disease - the flat flabby bubbles formed by erosion at the opening

Manifestations

Most often this disease is localized in areas of high friction (in the armpits, in the scrotum, under the breasts, on the inner thighs andneck) and manifests blisters turning into erosion.As a result of the merger with each other, the erosion transformed into intricately delineated erythematous plaques

aggravates the course of disease factors

The majority of patients with a diagnosis of Hailey-Hailey peak exacerbation occurs in the summer, as the increased body temperature and sweating seriously worsen the disease.Also contribute to relapse mechanical trauma, scalp electrodes during the procedure electrocardiogram, wearing a stiff collar, and women wearing bras with tight oppressive straps

From - the similarity of many of the symptoms, the disease Hailey-Hailey should be

clearly differentiated from other skin diseases: fungal andviral infection, pemphigus vulgaris, eczema, vegetating pemphigus, impetigo and Darier's disease .
from other dermatoses differentiate Hailey-Hailey allows that in the disease process has not involved the mucous membranes, and affects mostly intertriginous area

Treatment

Locally appointed :
- Fluorinated corticosteroid ointment
- fukortsin

In cases of prolonged treatment :
- neotigazon
- prednisolone (20-40mg per day)
- Antibiotics
In severe cases, treatment is prescribed methotrexate, thalidomide, dapsone, dermabrasion, PUVA - therapy, vitamin E, as well as laser treatmentcarbon dioxide.
With limited lesions that differ persistent current applied skin transplantation.

service physician recruitment is relevant only for the citizens of the Russian Federation

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