Ann Dermatol.  2017 Oct;29(5):626-629. 10.5021/ad.2017.29.5.626.

Histiocytoid Sweet Syndrome in a Child without Underlying Systemic Disease

• Yeom SD
• Ko HS
• Moon JH
• Kang MJ
• Byun JW
• Choi GS
• Shin J

• KMID: 2388917
• DOI: http://doi.org/10.5021/ad.2017.29.5.626

Abstract

Sweet syndrome (acute, febrile, neutrophilic dermatosis) is characterized by the acute onset of an eruption of painful nodules or erythematous or violaceous plaques on the limbs, face and neck. These symptoms are accompanied by fever. The diagnostic features include histopathological findings of dermal neutrophilic infiltration without leukocytoclastic vasculitis or peripheral blood leukocytosis. Sweet syndrome is associated with infection, malignancies, autoimmune disease, pregnancy, and drugs. Patients with Sweet syndrome demonstrate a complete and rapid response to systemic steroid administration. Recently, a distinct variant of Sweet syndrome was reported, termed "histiocytoid Sweet syndrome", in which the infiltration of myeloperoxidase-positive histiocytoid mononuclear cells are observed (in contrast to the infiltration of neutrophils). The other clinical features are similar to those of classic Sweet syndrome. Pediatric Sweet syndrome is uncommon, and the histiocytoid type is even rarer. To date, four cases of histiocytoid Sweet syndrome have been reported in children. Herein, we describe a case of histiocytoid Sweet syndrome in an otherwise healthy 10-year-old boy with no underlying systemic disease in whom non-steroidal, anti-inflammatory drug treatment was successful.

Keyword

Histiocytoid Sweet syndrome; Myeloperoxidase

MeSH Terms

Autoimmune Diseases
Child*
Extremities
Fever
Humans
Leukocytosis
Male
Neck
Neutrophils
Peroxidase
Pregnancy
Sweet Syndrome*
Vasculitis
Peroxidase

Figure

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  Fig. 1 Nontender nonpruritic erythematous macules and nodule

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  Fig. 2 (A, B) Infiltration of perivascular lymphocytes and large mononuclear cells with kidney shaped nuclei (arrow) in the dermis (H&E; A: ×40, B: ×400). (C, D) The presence of myeloperoxidase (MPO)-positive mononuclear cells in the dermis (MPO; C: ×40, D: ×400).

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