RESUMEN
Sarcoidosis occurs much more commonly in cohorts of connective tissue disease (1%) compared to the general population (0.01-0.04%). We present a case of concomitant connective tissue disease and cutaneous sarcoidal granulomas and discuss whether the observed granulomas represent a reactive phenomenon or true sarcoidosis. Click here for the corresponding questions to this CME article.
Asunto(s)
Enfermedades del Tejido Conjuntivo , Sarcoidosis , Enfermedades de la Piel , Humanos , Enfermedades de la Piel/etiología , Sarcoidosis/complicaciones , Sarcoidosis/diagnóstico , Granuloma/etiología , Enfermedades del Tejido Conjuntivo/complicacionesAsunto(s)
Fármacos Dermatológicos , Eccema , Erupción Variceliforme de Kaposi , Humanos , Erupción Variceliforme de Kaposi/tratamiento farmacológico , Estudios Retrospectivos , Hospitalización , Corticoesteroides/efectos adversos , Glucocorticoides/efectos adversos , Eccema/tratamiento farmacológicoRESUMEN
Pityriasis rubra pilaris (PRP) is a rare skin disease which manifests as a psoriasiform dermatosis and palmoplantar keratoderma and has significant clinical-histopathological overlap with psoriasis. Recently, several case reports have demonstrated successful treatment of PRP with anti-IL7A and anti-IL12/anti-IL23 monoclonal antibodies. We report a case of atypical juvenile PRP definitively diagnosed during adulthood with presence of CARD14 mutation. This case demonstrates a dramatic improvement with ustekinumab and highlights the role of genetic testing in chronic disease of diagnostic uncertainty.