British Journal of Dermatology (1979) 100, 551.

Disseminated intravascular coagulation associated with toxic epidermal necrolysis (Lyell's syndrome) JAN KVASNICKA,* JAN REZAC,t JIRI SVEJDA4 HANA DUCHKOVA,§ F R A N T I S E K K A Z E , * PAVEL ZALUDj AND JOSEF RICHTER1I Departments of * Haematology, t Anaesthesiology, + Infectious Diseases, § Dermatology, and H Immunology, Regional Hospital, tJsti nad Labem, Czechoslovakia Accepted for publication 22 August 1978

SUMMARY

Evidence of disseminated intravascular coagulation was recorded in eight patients with toxic epidermal necrolysis (TEN)—Lyell's syndrome. Patients were treated with low doses of heparin in combination with the usual treatment of TEN, i.e. maintenance of fluid and electrolyte balance, systemic corticosteroids, antibiotics and aseptic dressings, in the Intensive Care Unit environment. It is suggested that the alteration of haemostasis and inter-related biological systems, such as activation of components of complement, kinins and immunoglobuiins, may affect the outcome of TEN.

The first complete description of toxic epidermal necrolysis (TEN) was presented by Lyell (1956)The skin manifestations are the most dramatic but there is evidence for a multisystem involvement (Hoigne, 1970; Stich, 1970; Krumlovsky et al., 1974; Misgeld, 1974; Schopf et al., 1975). A histopathological study of TEN by Braun-Ealco (1970) showed, among other findings, an eosinophilic necrosis in the epidermis ranging from a coagulation to a coliquative necrosis. In the arterioles between the dermis and subcutis fibrin deposits, aggregates of erythrocytes and initial thrombi were detected. On the other hand, Rockl & Winkler (1970) described petechial haemorrhages, and Niederle (1968) reported massive, even lethal bleeding. These changes in the microcirculation and in haemostasis are best explained by the syndrome of disseminated intravascular coagulation (DIC) during the evolution of TEN (Macotela-Ruiz, 1972). In our observation of eight patients with TEN signs of DIC in the coagulation tests (Wolf, 1973) were found to be present to a lesser or greater extent, depending on the severity of the clinical condition and the time of the coagulation investigations. Correspondence: Dr Jan Kvasnicka, Department of Haematology, Regional Hospital, Pasteurova 9, 400 43 Xjsti nad Labem, Czechoslovakia. 0007-0963/79/0500-0551 $02.00 © 1979 British Association of Dermatologists

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J.Kvasniika et al. PATIENTS AND METHODS

During the years from 1974-1978 a total of thirteen patients with TEN were admitted to our hospital. Eight of them were treated in the Intensive Care Unit (ICU). The following investigations were performed: platelet counts with phase contrast microscopy (normal range 130-370x10'/!). Whole blood clotting—Lee White time (normal range 6-9 min). One stage prothrombin time (PT) with percentage of 'prothrombin activity' from dilution curves (normal range 80-100%). Activated partial thromboplastin time (APTT, normal range 28-35 s) as described by Colman, Oxley & Giannusa (1970). Euglobulin clot lysis time (normal value 2-5 h and above). Quantification of fibrinogen (Fbg, normal range 20-4-0 g/1) using a modified method of Ratnoff & Menzie (1951). Ethanol gelation test according to Godal & Abildgaard (1966). Detection of fibrinogen degradation products (FDP) in serum, at first by counterelectrophoresis (CEP) (Lewis, Wilson & Brandon, 1972) with specific anti-human fibrinogen serum, anti-fibrinogen split product D and E serum, manufactured by Behringwerke, Hoechst, Marburg-Lahn, West Germany, and later with Rapid latex test using Thrombo-Wellcotest'^'^ and with a tanned red cell haemagglutinationinhibition immunoassay (HAI, normal range 4-9+ 2-8 /(g/ml) using Wellcome FDP Kit'^^, manufactured by Wellcome Reagents Ltd, Beckenham, England. CASE REPORTS

Case I

A 5-year-old girl was admitted with TEN following administration of sulfamethoxydiazine on 18 April 1975. Her lips were bleeding, her temperature 39°C. Laboratory results are presented in Table i, coagulation studies in Table 2. TABLE I. Laboratory results in patients with TEN Case No. I 2

3 4 5 6 7 8

SGOT (iu/1) 60 105 825 300

1550 18 0 II-7 153 0

SGPT (iu/1)

Urea (mg/dl) Hb (g/dl)

Hct

WBC X io'/l 19 8

125

28

285 3150 18 0

53

10-4 84

033 033

24

10-1

III

032 038

140

54

12-8

190

32 29

17 5 3190

38 35

115 105

039 039 039

113 126 92 108

4650

121

032

236 69

She received treatment as follows: heparin 2000 iu three times daily s.e. (23-25 April), lincomycin, hydrocortisone, gamma-globulin and the necessary fiuid therapy with all aseptic precautions. After administration of heparin her urinary output rose from 630 ml to 1370 ml/24 h- On April 25 heparin was discontinued because of increasing bleeding from her lips, but the skin lesions showed signs of improving. By May 4 re-epithelialization was noted and the patient was discharged from the ICU. Case 2

A 21-year-old pregnant woman gave a past-history of pulmonary tuberculosis. Following admission to the maternity department for confinement it was deemed necessary to treat her prophylactically

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Disseminated intravascular coagulation associated with toxic epidermal necrolysis (Lyell's syndrome).

British Journal of Dermatology (1979) 100, 551. Disseminated intravascular coagulation associated with toxic epidermal necrolysis (Lyell's syndrome)...
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