Cutaneous manifestations of hemophagocytic lymphohistiocytosis.
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.


METHODS
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.


CONCLUSIONS
Awareness of cutaneous involvement can assist in the initial diagnosis of hemophagocytic lymphohistiocytosis and potentially signify recurrences.
Morrell
Dean S
DS
University of North Carolina, Department of Dermatology, 3100 Thurston-Bowles CB#7287, Chapel Hill, NC 27514, USA. morrell@med.unc.edu
Pepping
Marie A
MA
Scott
J Paul
JP
Esterly
Nancy B
NB
Drolet
Beth A
BA
D000971:Antineoplastic Combined Chemotherapy Protocols / Q000008:administration & dosage*; D001707:Biopsy, Needle; D003907:Dexamethasone / Q000008:administration & dosage*; D005260:Female; D005500:Follow-Up Studies; D015616:Histiocytosis, Non-Langerhans-Cell / Q000188:drug therapy* / Q000473:pathology*; D006801:Humans; D007150:Immunohistochemistry; D007231:Infant, Newborn; D012720:Severity of Illness Index; D012878:Skin Neoplasms / Q000188:drug therapy* / Q000473:pathology*; D016896:Treatment Outcome
D003907:Dexamethasone