Cutaneous Findings in Hemophagocytic Lymphohistiocytosis

• Published in: Dermatology (Basel) Vol. 230; no. 3; pp. 234 - 243
• Main Authors: Zerah, Michele L., DeWitt, Christine A.
• Format: Journal Article
• Language: English
• Published: Basel, Switzerland S. Karger AG 01.01.2015
• Subjects: Adolescent; Adult; Child; Child, Preschool; Dermatology; Disease; Female; Fungal infections; Humans; Infant; Infant, Newborn; Lymphatic system; Lymphohistiocytosis, Hemophagocytic - complications; Male; Middle Aged; Original Paper; Retrospective Studies; Skin Diseases - diagnosis; Skin Diseases - etiology; Young Adult; Stevens-Johnson syndrome; Hematologic malignancies; Neutrophilic dermatosis; Immunodeficiency; Viral; Erythema multiforme; Fungal infections; Bullous
• ISSN: 1018-8665; 1421-9832
• DOI: 10.1159/000368552

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.