Heliotrope-like manifestation of adult-onset Still disease with macrophage activation syndrome: A case-based review.

Adult-onset Still disease (AOSD) has been typically associated with an evanescent skin rash that appears during febrile episodes. Subsequently, reports of a more persistent rash have appeared in the literature, referred to as the atypical rash of AOSD. The atypical nonevanescent rash can be usually divided into dermographism-like, lichenoid, and dermatomyositis-like lesions. Some authors have suggested that AOSD with the atypical rash could be severe, with a poor prognosis. We describe the case of a Japanese woman with AOSD characterized by persistent pruritic lesions resembling those observed in heliotrope manifestation of dermatomyositis. We conducted a literature review of clinical cases of AOSD on MEDLINE and the Web of Science. We identified nine cases of atypical rash of the eyelids, heliotrope-like manifestation of AOSD in addition to our case. All nine patients were female and they had a mean age of 39.3 ± 2.8 years. Four (44.4%) patients had macrophage activation syndrome (MAS) or disseminated intravascular coagulation (DIC) as complications and our case was the only one associated with both MAS and DIC. When a clinician encounters a female patient with heliotrope-like rash resembling those observed in dermatomyositis, the underrecognition of the skin manifestations may result in delayed diagnosis of AOSD. We believe that physicians should identify this type of cutaneous lesion to diagnose AOSD earlier and administer adequate treatment. Although the contribution of tocilizumab to the occurrence of MAS has not been determined, careful observation should be considered during tocilizumab therapy in patients with active AOSD.