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

Affiliations
  • 1Department of Dermatology, Seoul St. Mary's Hospital, College of Medicine, The Catholic University of Korea, Seoul, Korea. yymmpark6301@hotmail.com

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

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

  • 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).

  • 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.

  • 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).


Reference

1. Morrell DS, Pepping MA, Scott JP, Esterly NB, Drolet BA. Cutaneous manifestations of hemophagocytic lymphohistiocytosis. Arch Dermatol. 2002; 138:1208–1212.
Article
2. Gupta S, Weitzman S. Primary and secondary hemophagocytic lymphohistiocytosis: clinical features, pathogenesis and therapy. Expert Rev Clin Immunol. 2010; 6:137–154.
Article
3. Fardet L, Galicier L, Vignon-Pennamen MD, Regnier S, Noguera ME, de Labarthe A, et al. Frequency, clinical features and prognosis of cutaneous manifestations in adult patients with reactive haemophagocytic syndrome. Br J Dermatol. 2010; 162:547–553.
Article
4. Henter JI, Horne A, Aricó M, Egeler RM, Filipovich AH, Imashuku S, et al. HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer. 2007; 48:124–131.
Article
5. DeWitt CA, Buescher LS, Stone SP. Cutaneous manifestations of internal malignant disease: cutaneous paraneoplastic syndromes. In : Goldsmith L, Katz S, Gilchrest B, Paller A, Leffell D, Wolff K, editors. Fitzpatrick's dermatology in general medicine. 8th ed. New York: McGraw-Hill;2012. p. 1880–1892.
6. Drago F, Romagnoli M, Loi A, Rebora A. Epstein-Barr virus-related persistent erythema multiforme in chronic fatigue syndrome. Arch Dermatol. 1992; 128:217–222.
Article
7. Valeyrie-Allanore L, Raujear JC. Epidermal necrolysis (stevens-Johnson syndrome and toxic epidermal necrolysis). In : Goldsmith L, Katz S, Gilchrest B, Paller A, Leffell D, Wolff K, editors. Fitzpatrick's dermatology in general medicine. 8th ed. New York: McGraw-Hill;2012. p. 439–446.
Full Text Links
  • AD
Actions
Cited
CITED
export Copy
Close
Share
  • Twitter
  • Facebook
Similar articles
Copyright © 2024 by Korean Association of Medical Journal Editors. All rights reserved.     E-mail: koreamed@kamje.or.kr