Case report: Localized Langerhans cell histiocytosis in caruncle

The caruncle was the site of an isolated Langerhans cell histiocytosis; complete surgical excision was achieved.

Issue: November 1, 2006

ByMark R. Levine, MD, FACS

ByKyu Mee Kay, MD

A 31-year-old man presented with a small, whitish-yellowish painless nodule in his right caruncle. He reported that his symptoms had started 2 weeks before his visit, and there had been no change in the size of the nodule since that time.

He had previously been treated with topical steroids, under the diagnosis of caruncular inflammation, by a local ophthalmic clinic, but the treatment failed to reduce the size of the nodule.

A solitary, yellowish, 4-mm-by-4-mm nodule located in the caruncle.

Focal collection of monocytic cells along with other inflammatory cells, typically eosinophils (hematoxylin-eosin, original magnification ×100).

Immunohistochemistry demonstrating positive staining for CD1a (original magnification ×200).

Images: Kay KM

Physical examination was unremarkable, except for a non-tender, swollen protrusion in the right caruncle. It was small (4 mm by 4 mm) and round, and it appeared soft (Figure 1). No limitation of eyeball movement, pain or visual disturbance associated with the nodule was detected. Also, there was no palpable lymph node enlargement.

A presumed diagnosis of sebaceous cyst of the caruncle was made, and excisional biopsy was planned.

During excision, the nodule showed isolated existence and no connection with inner structures of the orbit such as orbital fat, bone or surrounding soft tissues. The inner content of the nodule was noncystic and had an appearance of soft tissue.

Histological examination of the nodule revealed Langerhans cell histiocytosis positive for CD1a antigen and S-100 protein (Figure 2a-b).

After complete excision, there was no evidence of recurrence for 21 months.

Langerhans cell histiocytosis

Langerhans cell histiocytosis (LCH), first coined as “histiocytosis X,” is a benign, proliferative disorder of activated Langerhans cells that affects children more often than adults. It describes a broad spectrum of diseases, including disseminated LCH (also known as Letterer-Siwe disease), multifocal LCH (also known as Hand-Schüller-Christian syndrome) and unifocal LCH.

Localized LCH, also known as eosinophilic granuloma, is the most benign form. It occurs in about 70% of all cases of LCH. This condition is characterized by lesions containing lymphocytes, granulocytes, monocytes, eosinophils and Langerhans cells. Diagnosis is made by immunohistochemical demonstration of characteristic surface antigen (ie, CD1a) or the electron microscopic demonstration of Birbeck (Langerhans) granules.

Orbital involvement is uncommon, and the overall incidence of such involvement is estimated at 20% of all cases. It usually appears as an isolated form, mainly involving the superior-temporal quadrant, and it is associated with an osteolytic defect of the orbital roof.

A literature review showed few case reports of isolated orbital LCH limited to the orbital bones. Besides bony lesion, ocular involvement includes the eyelids, the uvea, the cornea and the optic disc. A case of epibulbar conjunctival nodule was reported as a symptom of LCH, but it was actually a part of a multifocal lesion.

For the treatment of localized LCH, there are several choices, such as curettage, intralesional steroids and complete surgical removal. We performed excision only, and no recurrence was found until postoperative 21 months.

This case, to our knowledge, represents the first case showing the caruncle as a site of isolated Langerhans cell histiocytosis not associated with orbit followed by complete surgical excision.

For more information:

Kyu Mee Kay, MD, is a clinical fellow at Korea University Guro Hospital in Seoul, Korea. She can be reached at 26300 Seville Drive, #111, Beachwood, OH 44122 ; e-mail : winona93@naver.com.

Mark R. Levine, MD, FACS, is a clinical professor of ophthalmology in the Department of Ophthalmology at Case Western Reserve University. He can be reached at University Suburban Health Center, 1611 South Green Road, Suite 306A, South Euclid, OH 44121; 216-291-9770; fax: 216-291-0550.

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