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Bilateral conjunctival Splendore-Hoeppli phenomenon associated with juvenile xanthogranuloma

      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.
      • Hussein M.R.
      Mucocutaneous Splendore-Hoeppli phenomenon.
      Hypereosinophilic syndrome and allergic conjunctival granulomas are noninfectious disorders that may also be associated with Splendore-Hoeppli phenomenon.
      • Hussein M.R.
      Mucocutaneous Splendore-Hoeppli phenomenon.
      • Read R.W.
      • Zhang J.
      • Albini T.
      • Evans M.
      • Rao N.A.
      Splendore-Hoeppli phenomenon in the conjunctiva: immunohistochemical analysis.
      In most conjunctival lesions, however, the definitive causative agent cannot be identified.
      • Read R.W.
      • Zhang J.
      • Albini T.
      • Evans M.
      • Rao N.A.
      Splendore-Hoeppli phenomenon in the conjunctiva: immunohistochemical analysis.
      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.
      Figure thumbnail gr1
      Fig. 1Biomicroscopic view of the superior conjunctiva of the left eye showing irregular, yellow-white tumefactions.
      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.
      Figure thumbnail gr2
      Fig. 2Histopathologic view of the specimen. A, Strongly eosinophilic amorphous material surrounded with epithelioid cells and multinucleated giant cells (hematoxylin and eosin [HE] staining, original magnification ×100). B, Splendore-Hoeppli material seen inside the giant cells (HE staining, original magnification ×200).
      The Splendore-Hoeppli phenomenon is characterized by an amorphous, eosinophilic substance surrounded by multinucleated giant cells, lymphocytes, epithelioid histiocytes, and eosinophils.
      • Hussein M.R.
      Mucocutaneous Splendore-Hoeppli phenomenon.
      • Read R.W.
      • Zhang J.
      • Albini T.
      • Evans M.
      • Rao N.A.
      Splendore-Hoeppli phenomenon in the conjunctiva: immunohistochemical analysis.
      This material is usually composed of either eosinophilic major basic protein or antigen–antibody complexes, possibly deposited against an infectious agent.
      • Hussein M.R.
      Mucocutaneous Splendore-Hoeppli phenomenon.
      • Read R.W.
      • Zhang J.
      • Albini T.
      • Evans M.
      • Rao N.A.
      Splendore-Hoeppli phenomenon in the conjunctiva: immunohistochemical analysis.
      Splendore-Hoeppli phenomenon in a patient with juvenile xanthogranuloma has not been previously reported.
      Juvenile xanthogranuloma is a self-limited benign disorder of early childhood characterized by a solitary, red or white cutaneous papule or nodule, and it belongs to non-Langerhans cell histiocytoses.
      • Janssen D.
      • Harms D.
      Juvenile xanthogranuloma in childhood and adolescence: a clinicopathologic study of 129 patients from the Kiel pediatric tumor registry.
      Histopathologically, proliferated histiocytic cells of CD14+, CD68+, fascin+, and factor XIIIa+ dendrocyte phenotype form the lesions.
      • Berres M.L.
      • Allen C.E.
      • Merad M.
      Pathological consequence of misguided dendritic cell differentiation in histiocytic diseases.
      The characteristic lipid-laden Touton giant cells have a macrophage morphology.
      • Berres M.L.
      • Allen C.E.
      • Merad M.
      Pathological consequence of misguided dendritic cell differentiation in histiocytic diseases.
      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.
      • Berres M.L.
      • Allen C.E.
      • Merad M.
      Pathological consequence of misguided dendritic cell differentiation in histiocytic diseases.
      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.
      • Pinto N.
      • Hirst L.
      • Whitby M.
      Conjunctival and episcleral Splendore-Hoeppli phenomenon.
      Regression either spontaneously or disappearance of other lesions after surgical excision of 1 lesion was also occasionally observed.
      • Read R.W.
      • Zhang J.
      • Albini T.
      • Evans M.
      • Rao N.A.
      Splendore-Hoeppli phenomenon in the conjunctiva: immunohistochemical analysis.
      • Orhan D.
      • Kiratli H.
      • Kocabeyoğlu S.
      Conjunctival Splendore-Hoeppli phenomenon in a 3-year-old child.
      Recently successful outcome was reported after use of topical cyclosporine A for 18 months.
      • McGrath L.A.
      • Whitehead K.
      • Lee G.A.
      Topical ophthalmic use of cyclosporin A for Splendore-Hoeppli phenomenon.
      Our experience with the patient with bilateral involvement supports the observations that the Splendore-Hoeppli phenomenon is a self-limited disorder that may completely resolve spontaneously.

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