Fever with persistent flagellate erythema in a primigravida: a rare presentation of adult-onset Still’s disease

Abstract

We describe a case of a 25-year-old primigravida, who presented to the emergency department with fever, arthralgia and erythematous maculopapular eruption. There was confluent violaceous macular erythema on the arms, v-area of the neck and upper back with periorbital oedema, mimicking dermatomyositis. There was flagellate erythema on the back. Skin biopsy and systemic investigations helped to rule out dermatomyositis. A final diagnosis of adult-onset Still’s disease with atypical cutaneous manifestations was made. This atypical variant is associated with a worse prognosis. Early recognition of this clinical variant can be life-saving for the patient.

Flagellate erythema with relapsing fevers and arthralgia

“…Moreover, flagellate erythema may serve as a poor prognostic sign in AOSD conferring longer chronicity, more internal organ complications, resistance to therapy, possible underlying malignancy, and higher mortality 4,5 . Thus, early recognition of this finding is critical in preventing morbidity and mortality 4,5 .…”

“…Moreover, flagellate erythema may serve as a poor prognostic sign in AOSD conferring longer chronicity, more internal organ complications, resistance to therapy, possible underlying malignancy, and higher mortality. 4,5 Thus, early recognition of this finding is critical in preventing morbidity and mortality. 4,5 Our patient was treated with colchicine and ibuprofen for the pericarditis, as well as 80 mg of intravenous methylprednisolone daily for 3 days followed by an 8-week oral prednisone taper starting at 60 mg daily.…”

“…4,5 Thus, early recognition of this finding is critical in preventing morbidity and mortality. 4,5 Our patient was treated with colchicine and ibuprofen for the pericarditis, as well as 80 mg of intravenous methylprednisolone daily for 3 days followed by an 8-week oral prednisone taper starting at 60 mg daily. At follow-up 1 month later, there was significant clinical improvement of all symptoms and laboratory abnormalities.…”

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

“…3,4 We also searched for "adult-onset Still disease" and "dermatomyositis" and reviewed the manuscripts of the relevant studies, which returned six articles. 1,[5][6][7][8][9] Based on this literature review, we identified nine cases of atypical rash of the eyelids, heliotrope-like manifestation of AOSD in addition to our case (Table 1). All nine patients were female, excluding one case in which details were not given, and they had a mean age of 39. ence in prevalence between males and females.…”

“…We conducted a literature review of clinical cases of AOSD on MEDLINE and the Web of Science, using search terms “adult‐onset Still disease” and “eyelid”, resulting in one article 3 and “adult‐onset Still disease” and “heliotrope”, resulting in two articles 3,4 . We also searched for “adult‐onset Still disease” and “dermatomyositis” and reviewed the manuscripts of the relevant studies, which returned six articles 1,5–9 . Based on this literature review, we identified nine cases of atypical rash of the eyelids, heliotrope‐like manifestation of AOSD in addition to our case (Table 1).…”