Dermatology - diseases of the skin

Lyell’s syndrome


Lyell's syndrome

Lyell’s Syndrome — toxic-allergic lesions of the skin and mucous membranes, often accompanied by changes in the internal organs and nervous system. Synonyms: LyeU-syndrome, epidermal necrolysis acute, necrolysis epidermal toxic, acute toxic epidermolysis, cutaneous allergic epidermal necrolysis, secretservice vascular allergic with a primary lesion of the skin and mucous membranes.

The Lyell’s syndrome — severe disease, with bullous lesions of skin and mucous membranes with detachment of the epidermis or the layer of epithelial cells, often due to the use of drugs. The Lyell’s syndrome – a severe, allergic, drug-induced disease that threatens the life of the patient, representing acute dermal-visceral pathology and intensive epidermal detachment and necrosis of the epidermis with formation of extensive blisters and erosions on skin and mucous membranes.

In General, clear definitions of the syndrome and Lyell’s syndrome and Stevens-Johnson is still there. Both of the syndrome are the most severe forms of drug drug reaction. Some believe the Lyell’s syndrome is the most severe variant of the syndrome Stevens-Johnson, however, according to other researchers, is pathogenetically different diseases and Lyell’s syndrome is a toxic manifestation of idiosyncrasy from the epithelial cells to certain drugs or their metabolites.

Causes of Lyell’s syndrome

Fully the causes of Lyell’s syndrome has not been studied. The Lyell’s syndromeoccurs as a result of taking some medications (drugs: sulfonamides, antibiotics, barbiturates), leads to necrosis of all layers of the skin. Therefore, it is important to remember that self-medication, especially with such strong means as antimicrobial, dangerous to health.

Pathogenesis of Lyell’s syndrome unclear. In cases associated with taking drugs, involve allergic mechanisms (types III and IV allergic reactions), where the drug is likely playing a role of hapten fixed to the proteins in the skin cells. Some researchers view as Lyell’s syndrome is the most severe manifestation mnogoformnuû erythema.

The appearance of erythematous patches on the skin and mucous membranes (erythematous stage) within 2-3 days turning into flabby thin-walled bubbles of irregular shape (bullous stage) with a tendency to merge, easily bursting with erosional extensive surfaces (deskwamatine stage). In the midst of disease affected surface resembles a burn with boiling water II–III degree

Positive symptoms Nikolsky (detachment of the epidermis) and Asbestos–Hansen (with pressure on the bladder increases its size is due to the detachment of the epidermis at the periphery of the bubble under the action of high pressure of its contents)

The lesion of the mucous membrane of the oral cavity, aphthous starting with and evolving to necrotic-ulcerative stomatitis

The defeat of the genital organs (vaginitis, balanoposthitis)

Hemorrhagic (with the transition to necrotizing) conjunctivitis — is the earliest manifestation of the disease • common symptoms Expressed with increasing intoxication caused by loss of fluid and protein through the affected eroded surface, impaired water-salt balance, the development of infectious lesions (often pneumonia, secondary infection of the skin), bleeding from the digestive tract including death.

Differential diagnosis spend with staphylococcal syndrome “burned” skin. The diagnosis of acute epidermal necrolysis is set on the basis of medical history (medication), clinical presentation (acute disease; severe General condition of the patient; the bubbles on the background of erythema; the symptom is detachment of the epidermis; the mucous membranes of the mouth, eyes, genitals), laboratory data, pathological studies (epidermal necrolysis, the formation of intra – and pogepegemana bubbles).

Lyell’s Syndrome should be distinguished from scalded skin syndrome .Lyell’s Syndrome it occurs mainly in adults, usually caused by drugs and is accompanied by a higher mortality. For the differential diagnosis performed needle biopsy of the skin with the study of frozen sections: the syndrome scalded skin exfoliates the Horny layer of the epidermis, when Lyell’s syndrome – entire epidermis entirely.

Symptoms of Lyell’s syndrome

The disease begins with a high body temperature to 39-40 °C, severe weakness, and sometimes pain in the throat. On the skin and mucous membranes occur the bubbles. With the appearance of the rash the patient’s condition is rapidly deteriorating. After opening the blistering on the skin remains large size moist wound surface. The disease may be accompanied by lesions of the internal organs — liver, kidneys, heart.

Symptoms and treatment of Lyell's syndromePrediction of treatment efficiency is poor in swargashram course, the late start of the active therapeutic interventions, as well as the accession of secondary infection. The mortality rate can reach 30-60%.

Treatment of Lyell’s syndrome

With the appearance of symptoms of Lyell’s syndrome requires urgent hospitalization of the patient. Cosmetics for removing toxins (detoxifying), gluco-corticoids, anti-allergic medicines.

Tactics

– Patients subject to compulsory hospitalization in the intensive care unit or the intensive care unit
– How burn patients are (preferably “burn tent”) in the most sterile environment to prevent exogenous infection
– Applied to development of the syndrome of drugs are subject to immediate cancellation.

Drug therapy

Treatment

The autopsy bubbles is not recommended . Irrigation erosion glucocorticoid aerosols (oxytetracycline+hydrocortisone, oksitsiklozol, etc.). Lubrication of water erosion R-Rami balinovac dyes; oozing erosion prescribe lotions with disinfectants (1-2% p-p of boric acid, R-RA Castellani). Apply creams, buttery mash, xeroform, solkoseril ointments ointments with GC (betamethasone+salicylic acid, methylprednisolone aceponate). If it affects the mucous membranes of the oral cavity shows astringent, disinfectant R-ry: an infusion of chamomile, R-R boric acid, borax, potassium permanganate for rinsing. Also applied water R-ry aniline dyes, R-R Boers in glycerine, egg white. Eye disease use of zinc or hydrocortisone drops.

Systemic therapy

GC: preferably in/methylprednisolone 0.25–0.5 g/day to 1 g/day in the most severe cases during the first 5-7 days followed by dose reduction. Detoxification and rehydration terapia. For the purpose of maintaining water, electrolyte and protein balance — infusion up to 2 liters of fluid per day: or reopoligljukin gemodez, plasma and/or albumin, isotonic R-R sodium chloride 10% R-R calcium chloride, R-R Ringera. Hypokalemia when used protease inhibitors (Aprotinin) . Systemic antibiotics may be indicated if secondary infection is under control the sensitivity to them microflora.

Prevention of Lyell’s syndrome

Prescription of medications with regard to their tolerability in the past, the rejection of the use of drug cocktails. It is recommended immediate hospitalization of patients with drug reaction associated with the overall phenomena, fever, and treatment with glucocorticoids in large doses. The survivors of Lyell’s syndrome, in 1-2 years it is necessary to limit immunisation, sun exposure, the use of tempering procedures.