Cutaneous manifestations of hemophagocytic lymphohistiocytosis. Arch Dermatol 2002 Sep;138(9):1208-12
Date
09/13/2002Pubmed ID
12224982DOI
10.1001/archderm.138.9.1208Scopus ID
2-s2.0-0036716898 (requires institutional sign-in at Scopus site) 70 CitationsAbstract
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 BAMESH terms used to index this publication - Major topics in bold
Antineoplastic Combined Chemotherapy ProtocolsBiopsy, Needle
Dexamethasone
Female
Follow-Up Studies
Histiocytosis, Non-Langerhans-Cell
Humans
Immunohistochemistry
Infant, Newborn
Severity of Illness Index
Skin Neoplasms
Treatment Outcome