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Treatment consists of clinical and nutritional support and management of pain and skin lesions. RESULTS: There is a shortage of scientific evidence related to the treatment of skin lesions in congenital EB, with most recommendations being based on expert opinions. Hydrofiber is indicated in most consensuses for wounds with some exudation and has been shown to be more absorbent than alginate.
In our experience, there was apparent improved control of pain, bleeding, and hypothermia with the use of hydrofiber, which has the advantage of not requiring daily changes and can remain on the wound for up to two weeks. Hydrofiber with silver is a treatment option for wounds in hereditary EB, without the need for daily dressing changes. EB is classified into four types, depending on the location of the mutated protein: simplex, junctional, dystrophic, and Klinder syndrome.
There are several subtypes according to the mutation that occurred. The laboratory diagnosis of EB is made by skin biopsy, immunofluorescence antigen mapping, electron microscopy, and mutation analysis1. More than different mutations involving 14 structural genes have been described. The mutations cause downregulation of proteins responsible for dermoepidermal adhesion, leading to the formation of blisters on a uniform cleavage plane. The severity of the disease will depend on the protein mutated and the degree of mutation2.
EB is a rare disease, with an estimated prevalence of 8. There is no racial or geographical preference3. The management of EB lesions is a challenge for physicians owing to the complexity and variety of its manifestations.
Plastic surgery has an important role in the treatment of complex wounds in EB. OBJECTIVE The objectives of this study were to review the general treatment and management of cutaneous lesions in EB, evaluate the indications and effectiveness of hydrofiber with silver, and report the experience of two consecutive patients with EB treated at our institution.
RESULTS General Recommendations If a neonate is suspected to have an EB, then one should immediately identify the type of lesions by performing a mapping of the affected areas and photographic documentation.
Calculation of the affected surface can be estimated using the same methods used for burned patients4. Regular follow-up of the patient should be performed with evaluation of the entire body in search of new lesions, including the scalp, external ear, oral cavity, and genital and anal regions5. Some general care should be performed with neonates: protection of the bony prominences; avoidance of using an incubator, since heat can lead to the formation of blisters; use of cord ligation rather than umbilical clamps; and avoidance of excessive nasal and oropharyngeal aspiration; however, if it is extremely necessary, soft, low pressure catheters and cushion below the pressure devices may be used; further, adhesives from the electrodes may be removed and fixed with non-adherent bandages.
Clothing should be easy to wear and not contain sewing. Diapers should not contain elastics; those with Velcro can prevent adhesive parts from sticking to the skin.
Avoid excessive manipulation of the neonate, and when transportation is required, the neonate should be held with one hand of the caregiver behind the neck and another on the buttocks, while avoiding excessive friction.
In breastfeeding infants, apply paraffin in the areola and breast to avoid trauma by the search reflex 5. Bathing can be done with saline solution or water. Acetic acid at a concentration of 0. The bath water should be warm. For heavily infected lesions, 0. For crustal lesions, emollients or oils may be used.
The frequency of the bath should be individualized according to the type and dressing used7,8. Keratolytic agents, such as urea and salicylic acid may be used to treat palmoplantar hyperkeratosis; however, special care should be taken in young children In addition, nasal lubrication should be performed constantly with products containing vitamin E or simply vaseline Thus, nutritional support should be initiated early, especially in severe cases of EB. Oral or intravenous iron supplementation is indicated in cases of microcytic anemia.
Combined use of iron with erythropoietin was proposed in an article Some other vitamins, such as vitamins A, C, D, and E, zinc, calcium, carnitine, and selenium can be replenished after evaluation and individualization for each case Enteral nutrition by probe should not be used for long periods, as this can cause erosion in the esophagus and oropharynx.
When necessary, the probes should be soft and small. Maximizing nutrition is of vital importance to promote growth and development in children, besides optimizing the healing of lesions In some cases, gastrostomy should be indicated to maintain adequate nutritional intake; however, in severe cases as in junctional EB type Herlitz, gastrostomy is not indicated in the palliative context6.
Low levels of hemoglobin in patients with EB are related to delayed healing of the lesions. Oral supplementation of iron can be performed with or without erythropoietin18, In older children and adults, it can be treated with antihistamines, such as hydroxyzine, loratadine, and cetirizine. In persistent pruritus, there is a report of treatment with ondansetron and low doses of gabapentin Pain control should be a part of the daily care of the neonate since in hereditary EB, pain is present from birth.
Care should be individualized for each patient, and it is necessary to consider the type of pain. Measurement of pain intensity is performed by scales for age. Pain can be acute, chronic, or neuropathic. The psychological component may be present in older children. The approach to pain must be both preventive, avoiding traumas, dissemination of blisters, and local infection, and therapeutic.
In conducting therapies, the treatment can be pharmacological or non-pharmacological6. Intense pain is linked to the manipulation of the patient, as in bathing and changing of dressings. Therefore, medications must be used before these procedures, and the type of medication will depend on the type of pain and age of the patient.
Topical anesthetics can be used to minimize pain during dressing and venous punctures; however, attention should be paid to toxic doses. Common painkillers, such as paracetamol are chosen in case of mild to moderate pain.
For more intense pain during handling, opioids can be used with or without hydroxyzine, and for control of anxiety and brief sedation, midazolam can also be used. In children, ketamine is rarely used in the changing of dressings For chronic pain, non-hormonal anti-inflammatory drugs may be used.
For neuropathic pain, pregabalin, gabapentin, or tricyclic antidepressants may be used together. For acute pain, local anesthetic blocks and botulinum toxin application are described for anal sphincter relaxation in case of erosions and fissures, in addition to paracetamol and opioids. Categories of wound dressings The choice of dressing varies according to the location of the lesions and availability of products6,9,10,23, Some products decrease the frequency of dressing change and reduce pain and manipulation, which consequently reduce the risk of blister formation and infection.
Non-adhesive dressings reduce pain at the time of their removal. In contrast, some systematic reviews showed only small advantage of advanced dressings hydrogels, hydrofibers, and foam when compared with gauze soaked in paraffin for chronic ulcers not related to EB Antibiotics and antiseptics are useful and have a proven role in treating superficial infection in chronic ulcers.
Some dressings, such as hydrogel, hydrofiber, and polymer membranes are useful in the debridement of devitalized tissues. However, in case of multiple or deep lesions, surgical debridement with anesthesia may be necessary5. There are a large number of dressings in the market for the treatment of EB lesions; for each case, the indications should be individualized, since there is no single ideal dressing.
It features a semi-permeable membrane, which allows the drainage of exudates. Depending on the amount of exudate, foams can be used up to 7 days, but require continuous change of secondary dressing. It is indicated for wounds with little or no exudation. Hydrogels improve pain, itching, and discomfort. It is indicated for wounds with exudation, with or without association to calcium ions which promote hemostasis 5,6, It is relatively inexpensive and non-adherent.
Further, biosynthetic cellulose reduces pain and itching. When in contact with exudates, the hydrocolloid polymers are hydrated and constitute a lipidocolloid interface with petroleum jelly, promoting a non-adherent dressing. It is indicated for wounds with exudates and for protection of vulnerable areas5,6. Hydrofibers are indicated for exudative and critically colonized or infected wounds. Products containing silver should be used with caution, especially in children, because of its potential toxicity by absorption; further, serum levels should be dosed properly in case of prolonged use.
It has limited use in wounds with little or no exudation or with crusts5,6,9, Choice of dressing according to the characteristics of the lesions a Dry or slightly exudative wounds: 1 Non-adhesive silicone dressings or lipidocolloid plaques or 2 thin and soft layer of silicone polyurethane and hydrogels can be used. Hydrogels can be changed daily, while the other types can be changed every days,9, In addition, soft silicone sponges and polymeric membranes are indicated.
Some of these dressings need another secondary dressing, since they do not adhere well In the presence of the above criteria, some studies suggest swab collection and treatment according to cultures, while others indicate the use of hydrofiber, alginate, and antibiotics for this type of wound,9, In addition, topical corticosteroids of medium potency can be used 5,6,9.
Further, topical corticosteroids can be used in short periods The Division of Plastic Surgery was requested to collaborate in the treatment of these patients with complex wounds resulting from EB. The lesions affected the head, including the mouth, limbs, trunk, perineum, mucous membranes, and nail beds. Therefore, they had severe cases of the disease with poor prognosis and low survival rates. The lesions were very exudative. Figure 1. Wounds in the trunk and limbs of the patients with epidermolysis bullosa - right lateral view.
Figure 2. Wounds in the trunk and limbs of the patients with epidermolysis bullosa - left lateral view. Figure 3. Wounds in the trunk and limbs of the patients with epidermolysis bullosa - front view. In these cases, dressings were changed according to their saturation. Figure 4.
ERISIPELA E CELULITE – Sintomas, causas e tratamento
Antibiótico para Erisipela Bolhosa, Nome, Tratamento
Antibiótico para Erisipela Bolhosa, Nome, Tratamento