RESUMEN
Pityriasis rubra pilaris (PRP) is an uncommon cutaneous disease with disorder of keratinisation. Up to now, systemic retinoids like acitretin or isotretinoin seem to be the most effective therapeutic agents. However, no large trials on this rare disease have been published and no standardised treatment has been established so far. Recently, single case reports demonstrate beneficial effects of alitretinoin (9-cis retinoic acid) in patients with PRP. We performed a retrospective observational analysis of type I adult-onset patients with PRP (n = 5) treated with systemic alitretinoin in our department. Alitretinoin was highly effective in the treatment of PRP in 4 of 5 cases. PASI score was reduced significantly in the alitretinoin responders. We assume that alitretinoin could serve as an additional effective systemic treatment option for type I adult-onset PRP.
Asunto(s)
Queratolíticos/uso terapéutico , Pitiriasis Rubra Pilaris/tratamiento farmacológico , Tretinoina/uso terapéutico , Anciano , Anciano de 80 o más Años , Alitretinoína , Femenino , Humanos , Masculino , Persona de Mediana Edad , Pitiriasis Rubra Pilaris/diagnóstico , Inducción de Remisión , Estudios Retrospectivos , Resultado del TratamientoRESUMEN
PURPOSE: Uveal melanoma (UM) is an orphan cancer of high unmet medical need. Current patterns of care and surveillance remain unclear as they are situated in an interdisciplinary setting. METHODS: A questionnaire addressing the patterns of care and surveillance in the management of patients with uveal melanoma was distributed to 70 skin cancer centers in Austria, Germany and Switzerland. Frequency distributions of responses for each item of the questionnaire were calculated. RESULTS: 44 of 70 (62.9%) skin cancer centers completed the questionnaire. Thirty-nine hospitals were located in Germany (88.6%), three in Switzerland (6.8%) and two in Austria (4.5%). The majority (68.2%) represented university hospitals. Most patients with metastatic disease were treated in certified skin cancer centers (70.7%, 29/41). Besides, the majority of patients with UM were referred to the respective skin cancer center by ophthalmologists (87.2%, 34/39). Treatment and organization of follow-up of patients varied across the different centers. 35.1% (14/37) of the centers stated to not perform any screening measures. CONCLUSION: Treatment patterns of patients with uveal melanoma in Germany, Austria and Switzerland remain extremely heterogeneous. A guideline for the treatment and surveillance is urgently needed.