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Disease Of The Skin

Bullous epidermolysis - causes, treatment, photo

Epidermolysis bulldozing is a group of hereditary skin diseases, characterized by the formation of blistering on the skin and mucous membranes. Rash occurs due to minor injuries, pressure on the skin, or spontaneously.

Contents

  • 1 Causes
  • 2 Forms of the disease
    • 2.1 Simple congenital
    • 2.2 Dystrophic
    • 2.3 Malignant
  • 3 Diagnosis of
  • 4 Treatment methods
  • 5 Treatment of folk medicine methods
  • 6 Prevention and prognosis
  • 7 Photo

Causes of

Causes of epidermolysis bullousto the end not studied. This is an hereditary disease, in families it is always inherited only one type of epidermolysis bullous. As a result of pathology of development in newborns, there is an inferiority of elastic fibers of the skin.

Important! Some forms of bullion epidermis are inherited by the dominant type of inheritance, while others are recessive.

Forms of the Disease

The term epidermolysis bullous includes several forms of the disease, differing in symptoms, course, and prognosis.

Simple congenital

Bullous epidermolysis causes, treatment, photo

Locations of the disease are hands, knees, elbows.

Simple epidermolysis bullous is found in 1 out of 50,000 newborns. This disease was described as early as 1886.The type of inheritance is the autosomal dominant, to date, known families in which a simple epidermolysis bullous is diagnosed already in the eighth generation.

The main symptoms of a simple form of epidermolysis bullous:

  • The disease begins at the time of birth and shortly after it. Boys suffer more girls than girls.
  • Bubbles appear on areas of the skin that have undergone even slight pressure, friction or other mechanical effects. The bubbles are filled with serous fluid, their size may vary from spike head to walnut. The skin around the bubbles is not ignited.
  • The main locations of rashes are hands, knees and elbows, legs and feet.
  • For most newborns, patients with a simple form of epidermolysis bullous, the first traumatic factor is the very process of childbirth. As a result, on the skin, which is closely in contact with the birth paths, there is a rash.
  • Blisters are exposed naturally or artificially. After their permission, surface defects in the skin( scarring, tissue dystrophy) are not observed. On the spot of exposed bubbles may be temporary pigmentation.
  • In a simple form of bullous epidermis, mucous membranes are extremely rare, in about 2% of cases. Nails do not collapse. There may be increased sweating of the feet and hands( about a quarter of patients).
  • The general health of the child is not disturbed, mental, mental and physical development occurs in accordance with the age.
  • Some deterioration is indicated by the age of 8-12 months, when the child begins to crawl and walk in connection with an increase in the degree of exposure to the skin.
  • As a rule, the symptoms of the disease appear brighter in the summer than in the winter.
  • As an adolescent, as a rule, bullous epidermis manifestations weaken or disappear completely.
  • A simple form of bullous epidermis may be classified as a localized form, which is also called an annual form or Weber-Kokkein syndrome. This form of the disease can occur not immediately after birth, but in the first or second year of life. Rashes in the summer form of bullous epidermis are located only on the feet and hands. Bubbles often have blood-sulfur content. The deterioration of the state of patients is noted in the summer, the field of hot tubs or when wearing warm shoes.

    Dystrophic

    Dystrophic bullous epidermolysis has two forms - hyperplastic and polydisplastic.

    Bullous epidermolysis causes, treatment, photo

    This type of illness weakens to adolescence.

    Symptoms of hyperplastic form of bullous epidermis:

    • , the disease begins to appear either immediately from the moment of birth, or after 1-2 days;
    • rash appears after any mechanical effects on the skin;
    • may cause cicatricial changes after the bubbles have been resolved;
    • syndrome of Nikolsky( a tendency to detach epidermis layers after mechanical effects on the skin) in this form of bullous epidermis is negative;
    • can affect not only the skin, but also the mucus, in which, after the resolution of rash, marked dystrophic changes;
    • hair, nails and teeth do not undergo pathological changes;
    • does not suffer physical and mental development of the baby;
    • is declining until the adolescence.

    Symptoms of polydisplastic form of bullous dystrophic epidermolysis:

    • disease manifests itself from the moment of onset;
    • rashes are observed on the skin and mucous membranes, and their education is not always associated with mechanical effects; bubbles may appear spontaneously;
    • often bubbles have bloody content. After their autopsy, ulcers are formed, which are gradually delayed;
    • rashes can capture large areas of skin surfaces;
    • Symptom of Nikolsky in this form of bullous epidermolysis is positive;
    • is often anxious for patients with severe itching;
    • nails quickly diminish and disappear altogether;
    • , after healing of the ulcers, forms a scar tissue that can restrict the mobility of the joints;
    • in this form of bullous epidermolysis often occurs necrotizing tissues and involuntary rejection of body parts, most often, phalanges of the fingers;
    • in patients often observed dysplasia( underdevelopment of organs or tissues), hypothyroidism( hypoplasia of the hair), anomalies of the teeth;
    • for polydisplastic form of bullous epidermolysis is characterized by severe lesions of the mucous membranes of the internal organs:
    • general condition of patients is severe, there is a strong delay in physical, and sometimes, mental development. Reduced resistance to infections. The prognosis in this form of bullous epidermolysis is unfavorable.

    Malignant

    This form of bullous epidermis is deadly. Manifests immediately after birth, the appearance of a generalized rash in the form of bleeding bubbles. Rash covers the skin and mucous membranes, including mucous membranes of the esophagus, stomach, intestines.

    After the blister is opened, painful, healing ulcers are formed. Congenital nasal dystrophies and bone tissue are observed. The disease is usually complicated by the addition of a pyococcal infection, sepsis and ending with the death of the infant.

    Diagnosis of

    Diagnosis of bullous epidermis is based on observing a clinical picture and collecting a family history.

    Histological studies do not allow the differentiation of bullous epidermis with other varieties of duct dermatosis. For the diagnosis, it is necessary to conduct laboratory tests:

  • . To determine the content of porphyry( if present, congenital porphyria is possible).
  • Serological reactions( can diagnose congenital syphilis).
  • An analysis of the contents of blisters on the detection of eosinophils, which allows differentiating bullous epidermolysis and herpetiform dermatosis.
  • Treatment Methods

    There are currently no specific methods for treating bullous epidermis. When choosing a therapy it is necessary to pay special attention:

  • Prevention of traumatic skin lesions;
  • Warning for secondary infections.
  • Bullous epidermolysis causes, treatment, photo

    Breastfeeding is the main method of treatment for bullous epidermolysis.

    At an early age, when the child has not yet developed immune mechanisms, it is important to prevent the risk of developing sepsis. It is highly desirable to provide a breastfeeding baby.

    In severe forms of bullous epidermis, treatment with glucosteroid hormones is indicated. As a rule, prednisone is prescribed to 60 mg per day, as the state of improvement improves, the dose is reduced until complete cancellation.

    In addition, prescribe anabolic drugs, broad-spectrum antibiotics, plasma infusions.
    Important! In the behavior of the treatment, it is necessary to constantly monitor the condition of the blood, in order to prevent the development of anemia in a timely manner.

    External therapy of bullous epidermis is as follows:

    • bubbles are formed on both sides with sterile needles;
    • covers the bubbles are lubricated with antiseptic solutions - diamond green, methylene blue, eosin, fuchsin;
    • make applications using erosion or heliomycin ointment on erosion;
    • use ointments containing glucocosteroids and antibiotics;The
    • bandage and bandage should be avoided in order not to exert additional pressure on the skin;
    • has a good effect on physiotherapy - a general UV.

    Treatment of folk medicine methods

    In addition to the prescribed therapy for bullous epidermolysis, it is possible to use the methods of folk medicine:

  • The use of common baths with the use of decoction of oak bark, chamomile, stalks, turnips is good.
  • You can make baths using a solution of manganese or adding olive oil.
  • To lubricate the crust after bubble separation, use sea buckthorn oil.
  • Prevention and Prognosis

    Prognosis with a simple form of bullous epidermolysis is favorable. More severe forms of the disease do not allow to give positive predictions for recovery.

    The primary prevention of bullous epidermis is to carry out a perinatal diagnosis. In case of severe heredity( in the family there were cases of dystrophic or malignant form of bullous epidermis), it is recommended to conduct a histological examination of the fetal biopsy. Biopsy of tissues is carried out in the second trimester of pregnancy under the control of ultrasound.

    Photo

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