Ann Dermatol.  2015 Oct;27(5):608-611. 10.5021/ad.2015.27.5.608.

Preceding Annular Skin Lesions in a Patient with Hemophagocytic Lymphohistiocytosis

• Jun HJ
• Kim HO
• Lee JY
• Park YM

• KMID: 2171463
• DOI: http://doi.org/10.5021/ad.2015.27.5.608

Abstract

The cutaneous manifestations of hemophagocytic lymphohistiocytosis (HLH) are variable and nonspecific. A 42-year-old man presented with multiple annular, erythematous patches on the trunk for 3 months. Two months later, he presented with bullae along with high fever. The laboratory examination showed pancytopenia, hypertriglyceridemia, and hypofibrinogenemia. The bone marrow biopsy specimen showed an active hemophagocytosis. On the basis of these findings, a diagnosis of HLH was concluded. After five cycles of chemotherapy, his skin lesion completely resolved. Taking the results together, we suggest that annular skin lesion can be added to the list of cutaneous manifestations of HLH.

Keyword

Annular skin lesions; Hemophagocytic lymphohistiocytosis

MeSH Terms

Adult
Biopsy
Bone Marrow
Diagnosis
Drug Therapy
Fever
Humans
Hypertriglyceridemia
Lymphohistiocytosis, Hemophagocytic*
Pancytopenia
Skin*

Figure

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  Fig. 1 Multiple, variable-sized, annular or arcuate erythematous to purpuric and pigmented patches (A) on the trunk and (B) right thigh.

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  Fig. 2 Vacuolar changes and dyskeratotic cells in the epidermis and a dense perivascular and diffuse inflammatory cell infiltration in the dermis. Upper dermal edema, mild vascular ectasia, and endothelial swelling were also seen. The infiltrating inflammatory cells were mostly lymphohistiocytes and a few neutrophils and plasma cells (H&E; A: ×20, B: ×100, C: ×400).

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  Fig. 3 Superimposed multiple denuded lesions on the annular erythematous and purpuric patches, which had increased in number, (A) on the trunk and (B) legs, 2 months later.

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  Fig. 4 Epithelial denudation, lichenoid infiltration in the upper dermis, and perivascular inflammatory cell infiltration in the mid and deep dermis. Fibrotic dermal change was also seen (H&E; A: ×20, B: ×100, C: ×400).

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