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Cutaneous manifestations of hemophagocytic lymphohistiocytosis. Arch Dermatol 2002 Sep;138(9):1208-12 PMID: 12224982

Pubmed ID



BACKGROUND: Hemophagocytic lymphohistiocytosis is a rare, rapidly progressive, and potentially fatal disorder of activated histiocytes. The initial clinical presentation commonly includes fever, hepatosplenomegaly, and pancytopenia. Skin eruptions are described in up to 65% of patients. Information regarding the morphological features, configuration, and distribution of these eruptions is lacking and is typically reported as nonspecific and "maculopapular." The aim of this report is to better delineate the cutaneous manifestations of the disorder to assist in differentiating the process from other systemic diseases.

OBSERVATION: A case report of a neonate with hemophagocytic lymphohistiocytosis with generalized purpuric macules is described. The clinical features of 5 other patients with hemophagocytic lymphohistiocytosis at Children's Hospital of Wisconsin, Milwaukee, are summarized. Clinical images of 1 additional neonatal patient with hemophagocytic lymphohistiocytosis are presented as well. These observations demonstrate the varied cutaneous manifestations of hemophagocytic lymphohistiocytosis: erythroderma, generalized purpuric macules and papules, and morbilliform eruptions.

CONCLUSION: Awareness of cutaneous involvement can assist in the initial diagnosis of hemophagocytic lymphohistiocytosis and potentially signify recurrences.

Author List

Morrell DS, Pepping MA, Scott JP, Esterly NB, Drolet BA


John Paul Scott MD Professor in the Pediatrics department at Medical College of Wisconsin


2-s2.0-0036716898   48 Citations

MESH terms used to index this publication - Major topics in bold

Antineoplastic Combined Chemotherapy Protocols
Biopsy, Needle
Follow-Up Studies
Histiocytosis, Non-Langerhans-Cell
Infant, Newborn
Severity of Illness Index
Skin Neoplasms
Treatment Outcome
jenkins-FCD Prod-331 a335b1a6d1e9c32173c9534e6f6ff51494143916