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Clinical phenotype and pathological features in 26 DRESS cases: are there markers of prognostic significance?

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journal contribution
posted on 14.11.2012, 23:16 by Salvador J. Diaz-CanoSalvador J. Diaz-Cano, D Creamer, S Walsh

Drug reaction with eosinophilia and systemic symptoms (DRESS) describes a severe medication-induced adverse reac- tion which has cutaneous, haematological and solid organ manifestations. The cutaneous and histopathological features of DRESS are heterogeneous, and an attempt to classify the phenotype has not previously been made. We reviewed the cutaneous findings and skin pathology in a series of 26 patients with a diagnosis of DRESS, all of whom had liver involvement as the solid organ manifestation. In each case, the cutaneous clinical phenotype was defined by the predominant reaction pattern, and presence of secondary skin signs. A primary dermatopathological pattern was assigned to each case on review of skin biopsy. The extent of liver dysfunction, along with other markers of systemic involvement, was also recorded. The most common primary dermatosis was an urti- cated papular exanthem (16/26), followed by an atypical ery- thema multiforme-like (EM-like) eruption (4/26), an exfoliative erythroderma (3/26) and a macular morbilliform erythema (3/26). Secondary features included pustules (4/26), mucositis (6/26), facial oedema (5/26) and purpura (8/26). The predominant histological pattern seen was a superfi- cial, spongiotic dermatitis with a lymphocytic infiltrate (17/ 26) which largely corresponded with the clinical appearance of an urticated papular exanthem. A smaller number of cases demonstrated necrotic keratinocytes and basal cell vacuolation (9/26). This pattern correlated with the EM-like clinical pre- sentation. An attempt was made to correlate specific clinical dermatoses in DRESS with the degree of systemic involvement, as determined by the extent of liver dysfunction. Severe hepa- tic involvement was defined as aspartate aminotransferase (AST) > 3 · upper limit of normal ± signs of liver failure (disturbed clotting, encephalopathy and admission to liver ITU); mild-to-moderate liver involvement was defined as maximum AST < 3 · upper limit of normal, without signs of liver failure. According to the above criteria, 14/26 had severe hepatic involvement. All nine cases demonstrating necrotic keratinocytes and basal cell vacuolation on biopsy, including the five cases with an atypical EM-like presentation clinically, were in this group. Mean maximum AST in the severe group was 1257 iu L–1 (207–5183 iu L–1) compared with 98 iu L–1 (22–144 iu L–1) in the mild-to-moderate group. Four cases from the severe group were admitted to liver ITU; two of these patients developed fulminant liver failure and underwent emergency liver transplantation. Both cases subsequently died. This series demonstrates the clinicopathological features in 26 DRESS cases and indicates that an EM-like presentation and presence of necrotic keratinocytes on biopsy may correlate with a poor prognosis, as defined by severe hepatic involvement.

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