Chronic granulomatous dermatosis as a presenting sign of a lymphoepithelioid T-cell lymphoma (Lennert lymphoma)

Abstract

PRN sedating antihistamine 43 (44) 0 (0) PRN non-sedating antihistamine 35 (36) 5 (5) Daily sedating antihistamine 7 (7) 0 (0)Daily non-sedating antihistamine 11 (11) 27 (28) High dose non-sedating antihistamine 1 (1) 66 (67) Additional agents 23 (23) 28 (29) *Additional treatment strategies following specialist review were only employed in patients in whom the response to high dose antihistamines was incomplete.standard first line therapy of a regular, standard-dose, nonsedating antihistamine. 44% were treated with as-required sedating antihistamines, out of keeping with accepted best practice. Prescribing patterns after specialist review differed considerably from strategies utilised by referring primary care doctors. Non-sedating antihistamines were the main pillar of specialist management and were used at above licensed doses in 67% of cases. 29% of patients required therapy in addition to high dose non-sedating antihistamines, including 5% in whom immunosuppression was required. This data indicates compliance with stepwise EAACI/WAO guideline based management after specialist review. The application of guideline based management resulted in a good outcome in 78% of this previously severely affected cohort. 14% of patients had an adequate outcome, while a further 8% had no improvement despite appropriate management and the use of multiple agents. This retrospective study describes a cohort of patients with long-lived troublesome chronic spontaneous urticaria. Despite significant symptoms, patients were not managed in line with international guidelines prior to specialist review. The main specialist strategy was to prescribe antihistamines on a daily basis and, where required, to increase the dose to above licensed levels. This simple intervention improved outcomes for the majority of patients. The lack of employment of regular high dose antihistamines by primary care practictioners was particularly striking. Liason between primary care and regional specialists with the application of local innovations such as shared care guidelines could lead to more timely and effective intervention. 22% of patients remained symptomatic despite appropriate management using multiple agents. It is this treatment resistant group that is most likely to benefit from specialist input and access to emerging treatment strategies [6]. This study demonstrates poor implementation of EAACI/WAO guidelines for CSU among primary care practitioners. Given the resource limitations on some specialist services, education of primary care physicians and early application of guideline-based CSU management may be a cost efficient way of improving outcome for this neglected patient group.Disclosure. Financial support: none. Conflict of interest: none.