Cutaneous findings in hemophagocytic lymphohistiocytosis.

MedStar author(s):
Citation: Dermatology. 230(3):234-43, 2015.PMID: 25677716Institution: MedStar Washington Hospital CenterDepartment: DermatologyForm of publication: Journal ArticleSubject headings: *Lymphohistiocytosis, Hemophagocytic/co [Complications] | *Skin Diseases/di [Diagnosis] | Adolescent | Adult | Child | Child, Preschool | Female | Humans | Infant | Infant, Newborn | Male | Middle Aged | Retrospective Studies | Skin Diseases/et [Etiology] | Young AdultYear: 2015ISSN:
  • 1018-8665
Abstract: BACKGROUND: Cutaneous findings associated with hemophagocytic lymphohistiocytosis (HLH) remain largely undescribed in the literature, yet are substantial and correlative with disease course.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.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.OBJECTIVE: To catalog the clinical findings of cutaneous eruptions associated with HLH.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.All authors: DeWitt CA, Zerah MLFiscal year: FY2016Digital Object Identifier: Date added to catalog: 2016-01-13
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Journal Article MedStar Authors Catalog Article 25677716 Available 25677716

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

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.

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.

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

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.

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