Cutaneous findings in hemophagocytic lymphohistiocytosis.

BACKGROUND

Cutaneous findings associated with hemophagocytic lymphohistiocytosis (HLH) remain largely undescribed in the literature, yet are substantial and correlative with disease course.

OBJECTIVE

To catalog the clinical findings of cutaneous eruptions associated with HLH.

METHODS

We performed a retrospective chart review of patients meeting the criteria for HLH at two hospitals over 5 years. All patients meeting the criteria for HLH as defined by the HLH-2004 protocol were included.

RESULTS

Cutaneous lesions were categorized based on clinical presentations and histology. Lesions independent of immunocompromised state were observed, including pyoderma gangrenosum, panniculitis, morbilliform eruptions, Stevens-Johnson syndrome, atypical targetoid lesions and bullae. Histologic findings were non-specific.

CONCLUSION

Cutaneous eruptions as a consequence of HLH are variable in presentation and identified as a diagnosis of exclusion. Findings are both primarily and secondarily induced by altered immunity. Further study is needed to allow better understanding of the immunopathogenesis involved.