Ichthyosiform erythroderma of Broca: treatment, diagnosis, photos of patients

August 19, 2017 23:00 | Hereditary

In relation to the class of skin diseases, ichthyosymorphic erythroderma is a disease that affects the top layer of the epidermis with the formation of inflammation sites that are covered with blisters and many small dry scales.It is a congenital form of ichthyosis, which has a bullous form and is difficult to cure.To date, there is no precise data on the prevalence of this disease.

Features of the disease

Equally manifested in both men and women, their ichthyosiform erythrodermia affects the upper layers of the skin of the body, manifesting as constant peeling, the appearance of small dry scales and blisters.Transmitted as a hereditary factor, the disease begins as a result of gene mutation.An important role, according to the latest studies of the disease under consideration, is the increase in the level of n-alkanes.The mutation occurs in the gene that codes for the formation of epidermal trans-glutaminase.

The most commonly affected areas are axillary cavities, areas of large joints, inguinal fo

lds - in these areas lesions have a pronounced concentric shape. Inflammatory processes in the upper layer of the epidermis are accompanied by hyperkeratosis, with a different degree of pronounced acanthosis.There may also be an accumulation of horny plugs, especially at the mouth of the hair follicles, which hinders the growth and nourishment of the hair.There is a deterioration in the patient's hair.

Congenital bullous ichthyosiform erythroderma( photo)

Ihtioziformnaya-eritrodermiya

Classification of ichthyosiform erythroderma

Today, there are many different forms of erythroderma that have similar symptoms and manifestations.To clarify the diagnosis should be a number of additional laboratory studies that will make the most optimal treatment regimen.

This disease is classified according to the severity of its course:

  • of medium severity , in which only some areas are affected( mainly the axillary and anus grooves regions);
  • is a severe degree of lesion that is characterized by significant areas of skin damage.

There are also a number of syndromes, including ichthyosiform erythroderma:

  1. Syndrome of Netherton;
  2. syndrome Conradi-Hyunermann;
  3. Sjogren-Larsson syndrome;
  4. syndrome of accumulation of neutral fats;
  5. Tau syndrome.

Causes of

In the opinion of some dermatologists, the disease in question can arise due not only to a hereditary gene mutation, but also to the appearance of disturbances in the synthesis of tonofilaments, the inability to excrete platelets from epitheliocytes. Inheritance of this disease occurs in an autosomal dominant type.

Symptoms of

The manifestations of ichthyosiform erythroderma are very typical for the whole variety of ichthyosis forms, in which the upper layer of the epidermis is affected and symptoms such as:

  • appear, the skin feels tight;
  • on the skin surface, a large number of dry scales gradually form, which can have from light gray to dark brown hue;
  • the skin in the lesions acquires a bright color, the hair on it becomes less and less due to the constant clogging of the hair follicle's mouth with granules of dead skin;
  • is a frequent form of the disease - a congenital kind of disease in which the baby is already born in the damaged skin.Most often in this case, the affected areas of the face, trunk;On a body linearly located eruptions of a verrux character.Plantar and palmar keratosis can also be observed.

Since it is n-alkanes that are responsible for the process of solidification of the intercellular substance, in the event of disturbances in their production and accumulation, the brittleness and excessive hardness of the surface( horn) layer of the epidermis increase.

Diagnostics

  • Ihtioziformnaya-eritrodermiya For the preliminary diagnosis, an experienced dermatologist will have to examine the patient visually;however because of the considerable amount of ichthyosis species should analyze such parameters as the presence of the blood vessels in the dermis, the degree of atrophic hair follicles and sebaceous ducts, whether perifollicular and perivascular inflammatory infiltrates in the superficial layer of the skin.These data are typical for their ichthyosiform erythroderma.
  • Also used are diagnostic methods, such as antenatal methods.In the presence of clots of tonofilament, as well as the absence of complexes of tonofibrils, this disease can be diagnosed: in the skin of healthy people, they are absent in the cellular granular layer.Analysis of amniotic fluid also allow diagnosis during pregnancy: the presence in it of the epithelial cells with pyknotic nuclei and containing tonofilaments units, also indicate a high probability of ichthyosiform erythroderma in the fetus.

Treatment

Complete recovery of the disease under consideration is impossible today. However, the complex approach to treatment allows in a short time to a large extent to improve the overall skin condition, eliminate the active formation of dry scales on its surface.

therapeutic method

to improve skin dermatologist may be assigned the administration of drugs with a high content of vitamin A, which has a positive effect on the overall condition of the skin restores skin elasticity and firmness.

Vitaminotherapy perfectly helps to increase the level of immunity, which is also important in the development of ichthyosiform erythroderma.Complex Vitamins should be prescribed by a dermatologist, in their composition must be vitamin A.

medicamentous way

Using reitnoidov reduces skin cell rejection process that stops the peeling and moisturizers, especially after hygienic procedures will help restore softness, elasticityAnd elasticity.

Among other medicines in the treatment of the disease in question, particular attention is given to Acitretin, which is taken orally in an amount of 10-35 mg / day.Duration of treatment is 4 weeks, then a break should follow, then the treatment is repeated.To the keratolytic agents that help restore the skin condition and reduce the symptoms of the disease, the following should be attributed:

  • urea, ointment 2-11%, applied topically;
  • hepatoprotectors in combination with fortifying agents;
  • ointment Lactic acid( 3-6%), also applied topically until the appearance of the improvement.

The evaluation of the effectiveness of the treatment is based on the analysis of the skin condition, the reduction of clinical manifestations of the disease.

Erythroderma-Brock Wilson( photo)

Eritrodermiya-vilsona-broka

disease prevention

  • Since this type of dermatological diseases is inherited, couples who are planning a pregnancy, if they or their immediate family history of erythroderma differentCharacter should undergo a general examination and consult a dermatologist.
  • Also in order to maintain improved after the treatment, you should regularly perform hygienic procedures using soft exfoliating drugs, special gels for dry skin and ointments based on urea.

forecast

When a child is born with a congenital erythroderma should immediately apply the treatment of retinoids, which allows you to save his life. When diagnosing the disease in adulthood, to improve the quality of life, all the recommendations of a dermatologist should be followed, as well as the prescribed treatment.