Adult onset still's disease and prurigo pigmentosa: an unusual association and review of literature

ABSTRACT

This study was conducted in the Department of Medicine, Department of Dermatology, Al-Adan Hospital, Ministry of Health-Kuwait. This patient was hospitalized as a case of pyrexia of unknown origin. We report atypical skin eruption in this patient who fulfilled the criteria for Adult Onset Still's Disease [AOSD] despite the absence of the typical still's rash. Skin biopsy revealed prurigo pigmentosa [PP]. To elucidate this unusual association, we searched the literature for the cutaneous manifestations of AOSD. Kahori T, et al had described PP like lesions in a patient with AOSD in addition to the typical still's rash and it is the only case report that exists in English literature. In our case, except for still's rash, all the criteria of AOSD were present, the diagnosis was made after excluding all other possible causes i.e. infectious diseases, other rheumatic diseases, vasculitis and malignant disease. The objective for reporting this case and review of literature is to shed more light on the rare dermatological disorder Prurigo Pigmentosa [PP] and its unusual association with Adult Onset Still's Disease [AOSD]. Clinicians and dermatologist must be aware of this unusual association of PP and AOSD as the diagnosis of AOSD can be made in the absence of the typical still's rash but in the presence of other atypical cutaneous manifestations including PP