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

Christina S Thornton,Aurore Fifi-Mah,Michelle Schneider,Parham Minoo

doi:10.1186/s41927-019-0055-x

Christina S Thornton, Aurore Fifi-Mah + Show 2 more

Open Access

https://doi.org/10.1186/s41927-019-0055-x

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Journal: BMC rheumatology	Publication Date: Feb 8, 2019
Citations: 3	License type: open-access

Affiliation: University of Calgary, South Health Campus

Abstract

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

Highlights

Hemophagocytic lymphohistiocytosis (HLH) is a severe clinical entity associated with high mortality in the adult population
Conclusions: we highlight that a keen clinical acumen is required as misdiagnosis may lead to insufficient treatment with adverse clinical outcomes with the unique presentation of HLH from inaugural cases of Systemic Lupus Erythematosus (SLE)
While there have been several reports in the literature of HLH in adulthood associated with autoimmune conditions such as systemic lupus erythematosus (SLE), often these have been associated with disease flares

Summary

Introduction

Hemophagocytic lymphohistiocytosis (HLH) is a severe clinical entity associated with high mortality in the adult population. While there have been several reports in the literature of HLH in adulthood associated with autoimmune conditions such as systemic lupus erythematosus (SLE), often these have been associated with disease flares. We present here two cases of cutaneous lupus as the initial manifestation of HLH, a rare but clinically relevant entity.

Results

Conclusion