J Cutan Pathol 2008: 35: 694–695Blackwell Munksgaard. Printed in Singapore

Copyright # Blackwell Munksgaard 2008

Journal of

Cutaneous Pathology

Letter to the Editor

Touton-like giant cells in a Spitz’s nevus

To the Editor,Juvenile xanthogranuloma (JXG) is the most

common condition included in the clinical differen-tial diagnosis of Spitz nevus. The clinical resem-blance correlates with the symmetrical dome shapedinfiltrative appearance of both lesions, which iscomposed of a dense dermal infiltrate with largecells. Although the cytological detail of bothconditions is different, the cells share some featureslike an abundant cytoplasm, which for the most partlacks significant pigmentation. Dermal edema,telangiectasia and scattered inflammatory cells areequally noted in both conditions. Touton giant cells(TGC) are the prototypical sign of JXG. We reporta case of an otherwise unremarkable Spitz’s nevuswith classical TGC.

The patient is a 4-year-old boy who presented tothe pediatrician with a growing left arm lesion. Priorto excision the differential diagnosis of the derma-tologist was JXG vs. Spitz nevus. The lesion wasentirely excised. Sections showed a dome shapedlesion surmounted by a slightly acanthotic epider-mis. The papillary dermis was filled with nests andfascicles of large epithelioid to spindle shapedmelanocytes with sharp cytoplasmic boundariesabundant pale cytoplasm and open chromatin withprominent lavender nucleoli. Numerous multinucle-ated melanocytes were noted. Rare cells had thetypical wreath nuclear configuration with a periph-eral rim of cytoplasmic clear vacuoles (Fig. 1). Thedeep dermal component showed a gradient ofmaturation with the descent of the cells and lackof nuclear pleomorphism or atypia. The melano-cytes including TGC were positive for S100 proteinand MART-1 (Fig. 2). Since histiocytes can phago-cytize proteins of melanocytic origin, CD68 andfactor XIIIa immunohistochemistry were performedand the multinucleated cells were negative ruling outa histiocytic origin.

First described by Karl Touton �xanthelasmaticgiant cell’, TGC is a large lipid laden histiocyte withmultiple nuclei arranged in a circle with a centralsmall island of non-foamy cytoplasm.1 TGCs are

a characteristic feature of JXG. However, othercutaneous histiocytosic conditions like papularxanthoma, xanthelasma, necrobiotic xanthogranu-loma and occasionally Rosai-Dorfman’s disease2–9

Fig. 1. Touton giant cell with prominent vacuolization and focal

pigmentation of the cytoplasm. (hematoxylin and eosin 360).

Fig. 2. Mart-1 immunohistochemistry decorates the Touton giant

cell as well as the adjacent melanocytic nests.

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can display giant cells with the same cytomorphology.TGC have also been reported in Erdheim-Chesterdisease, a systemic histiocytosis with frequent involve-ment of brain, lungs, bones, heart and orbit.6,10–12 Inexceptional cases TGC have been reported in otherunexpected conditions like sarcoidosis or soft tissuesarcomas.13–15

We observed Touton-like giant cells in an other-wise unremarkable Spitz’s nevus. Multinucleatedcells are common in nevomelanocytic lesions andthe TGC appearance is simply a variant withabundant cytoplasmic vacuolization. This observa-tion expands the spectrum of skin lesions withpossible TGC. Awareness of this finding is importantbecause JXG and Spitz’s nevus can have a similarclinical presentation and are often included in thesame differential diagnosis.

Joan Guitart, MD andPedram Gerami, MD

Department of Dermatology, Northwestern UniversityFeinberg Medical School,

Chicago, IL, USAe-mail: j-guitart@northwestern.edu

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Letter to the Editor

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