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The Splendore-Hoeppli phenomenon is considered a rare and distinct local mucocutaneous inflammatory reaction to antigens of a vast variety of infectious agents including fungi, bacteria, and parasites, and to some foreign material such as silk sutures.
We describe a case of spontaneously regressed multiple bilateral conjunctival Splendore-Hoeppli lesions in association with juvenile xanthogranuloma of the scalp.
A 6-year-old male was brought by his parents for itchy eyes and small opaque nodules over both eyes that developed within 10 days. Six months previously, a red papular lesion had been removed from his scalp, which proved to be juvenile xanthogranuloma. On our examination, his visual acuity was 20/20 OU. There were bilateral, multiple, small, pale yellow, and irregular conjunctival nodules (Fig. 1). All were located in the superior half of the conjunctiva. The lesions were painless, soft, and mobile on palpation. Dilated episcleral vessels were also observed. The rest of the ocular examination was noncontributory. A full blood count and blood biochemistry tests were normal; no eosinophilia was detected. A week later, an excisional biopsy of 1 of these lesions was performed. However, at the time of the operation most of the lesions had already disappeared bilaterally. No treatment was offered and there has been no recurrence during follow-up.
Histopathologic examination of the specimen showed granulomas beneath the conjunctival epithelium. The granulomas were composed of multinucleated giant cells, lymphocytes, and epithelioid histiocytes surrounding bright eosinophilic amorphous material and cellular debris (Fig. 2A). Splendore-Hoeppli materials were observed inside the giant cells (Fig. 2B). No specific microorganism was identified. A systemic work-up in search of an infectious disease was noncontributory, and the stool tested negative for parasites.
The Splendore-Hoeppli phenomenon is characterized by an amorphous, eosinophilic substance surrounded by multinucleated giant cells, lymphocytes, epithelioid histiocytes, and eosinophils.
Juvenile xanthogranuloma has been observed in association with neurofibromatosis types 1 and 2 and juvenile chronic myelomonocytic leukemia, suggesting Ras pathway deregulation as a common pathogenetic basis.
Although juvenile xanthogranuloma and Splendore-Hoeppli phenomenon have distinctly separate histopathologic features, further observations are necessary to determine whether these conditions may share common immunopathogenic dysfunction at some point during their evolution. The coexistence of these disorders in our patient may also be merely fortuitous.
There is no established treatment for the Splendore-Hoeppli phenomenon. The use of topical or systemic corticosteroids is controversial because this was found to be of no benefit in many patients, as demonstrated in a patient in whom the Splendore-Hoeppli phenomenon developed under high-dose oral steroids and disappeared during withdrawal of the drug.