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Cutaneous Manifestations of Hemophagocytic Lymphohistiocytosis
Dean S. Morrell, MD;
Marie A. Pepping, MD;
J. Paul Scott, MD;
Nancy B. Esterly, MD;
Beth A. Drolet, MD
Arch Dermatol. 2002;138:1208-1212.
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.
From the Department of Dermatology (Drs Morrell, Pepping, Esterly,
and Drolet), and the Division of Pediatric Hematology and Oncology (Dr Scott),
Medical College of Wisconsin and Children's Hospital of Wisconsin, Milwaukee,
Wis.
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