Severe skin disease in lupus associated with hemophagocytic lymphohistiocytosis: case reports and review of the literature

• DOI: 10.1186/s41927-019-0055-x
• Publication Date: 2019-02-08T20:35:24Z
• Authors: Christina S. Thor...; Parham Minoo; Michelle Schneider; Aurore Fifi-Mah
• Keywords: Hemophagocytic Lymphohistiocytosis; myalgia; Presentation (obstetrics)

Hemophagocytic lymphohistiocytosis (HLH) is a severe clinical entity associated with high mortality in the adult population. HLH has been infections, malignancy and autoimmune conditions such as Systemic Lupus Erythematosus (SLE), however this often context of disease flare. Currently, there are limited reports inaugural SLE manifesting lack consensus on treatment management these patients.Here, we present two rare case cutaneous manifestation lupus HLH. Both patients presented sinister courses primarily rheumatologic complaints including malaise, arthralgia, myalgia biochemical abnormalities. were diagnosed result first presentation from lupus. A comprehensive literature review using PubMed database cases comprising keywords up to September 2017 was conducted, an emphasis cases.Ultimately, highlight that keen acumen required misdiagnosis may lead insufficient adverse outcomes unique SLE.