S Kumar, A Sood, B Badhu, M Lakhey, R Kulshreshtha
Department of Ophthalmology and Department of
Pathology, BP Koirala Institute of Health Sciences,
Dharan, Nepal

This is a report of unifocal langerhans cell histiocytosis presenting atypically as a fungating granuloma in a 12-year-old boy. There was a history of incision and drainage for a tender upper eyelid swelling mistaken for a lid abscess. An exuberant granulomatous growth developed at the site of the incision, which did not regress with antibiotics and anti-inflammatory agents. Incision biopsy and histopathological examination showed eosinophils and Langerhans cells, thus confirming the diagnosis. Treatment with intralesional steroids resulted in a remarkable and complete regression. Although upper lid eosinophilic granulomas are known to occur, this is a unique case on account of its presentation, which has not been reported before.

Key words: Eosinophils, Fungating, Granuloma, Langerhans, Steroids, Unifocal

Asian J Ophthalmol 2003;5(1):17-19.

Case Report


12-year-old boy presented with a fungating mass over the supero-temporal aspect of the right upper eyelid for 4 weeks (Figures 1 and 2). There was a history of a tender swelling on the upper lid, with no preceding history of a boil on the lid, trauma, fever, eyeball prominence, headache, or diminished vision. Further, the patient's past medical history did not reveal any episodes of any other masses on the face or body. The mass had been treated as a lid abscess, and was incised and drained. Three days postoperatively, an exuberant vascular mass developed at the site of incision and gradually increased in size despite treatment with oral antibiotics and anti-inflammatory drugs.

Figure 1. Fungating granuloma on right upper lid (antero-posterior view).	Figure 2. Fungating granuloma on right upper lid (lateral view).

At ocular examination, the vision was 6/6 in both eyes and no abnormality was detected in the anterior and posterior segments. There was a 3 cm x 4 cm fungating superotemporal mass, with an irregular, highly vascular surface. The mass wasnon-mobile, tender, with no pus discharge or associated lymph nodes. An X-ray ofthe skull showed an osteolytic lesion in theright frontal bone just above the superior orbital plate (Figure 3).

Figure 3. Lateral radiograph of skull showing osteolytic lesion.

Figure 4. Computed tomography of skull showing bony defect.

Incisional biopsy recorded infiltration by large numbers of eosinophils, histiocytes, and multinucleated giant cells (Langerhans cells) [Figure 5]. Routine haemogram was within normal limits. Absolute eosinophil count was as high as 9.01 x 10⁹/L (normal range, 0 to 0.45 x 10⁹/L).

Figure 5. Microphotograph showing eosinophils and langerhans cells.

Urine specific gravity was within normal range. Chest X-ray and ultrasonogram of the abdomen revealed no evidence of systemic involvement.

The patient was treated with intra-lesional triamcinolone acetate (0.5 ml of 40 mg/ml), repeated after 1 week. The lesion completely resolved, leaving only a small defect in the skin of the upper eyelid (Figures 6 and 7), which was subsequently repaired.

Figure 6. Complete post-treatment regression (antero-posterior view).	Figure 7. Complete post-treatment regression (lateral view).

Discussion


Langerhans cell histiocytosis (LCH) is a term used to describe histiocytic mass lesions that are unifocal or multifocal and generally originate at bony sites. These lesions involve mononuclear phagocytic cells and are characterised by proliferation and activation of macrophages.¹ The actual trigger is unknown but an immunoregulatory defect with decreased suppressor T cells has been postulated.² Histiocytosis X has been known to occur in 3 forms - eosinophilic granuloma, now known as LCH, Hand Schuller Christian (HCS) disease, and Letterer Siwe (LS) disease. Eosinophilic granuloma occurs in older children and young adults and has an excellent prognosis as was seen for this patient. Diabetes insipidus with bony defects and proptosis is the classic triad of HSC disease. Vital organ and bone marrow involvement is seen in LS disease, which affects infants and children younger than 2 years, and has a poor prognosis. For patients with localised orbital involvement, LCH has a distinct prediliction for the superotemporal orbital rim³ as was seen with this patient. Solitary LCH has commonly been reported to present as eyelid swelling,⁴ ptosis,⁵ or rapidly progressing proptosis.⁶ This patient presented with a fungating mass over the right upper eyelid, which has not been reported before. Due to the possibility of systemic involvement or progression to more disseminated forms of histiocytosis X, a thorough evaluation was performed. No evidence of systemic involvement was found, and the absence of proptosis suggested sparing of the orbit.

Other intraosseous histiocytic lesions considered were cholesteatoma⁷ and giant cell reparative granuloma.⁸ But since these were not associated with eosinophilia they were excluded for this patient. Eosinophilic lesions without bony defects raise the possibility of granulocytic sarcoma or paediatric pseudotumour.⁹ Dacryoadenitis is another condition which may mimic unifocal LCH when the histiocytic lesion breaks out of diploe and periorbita with resulting inflammation.⁹ The characteristic histopathological biopsy report, supported by the presence of lytic bone lesions on X-ray and CT confirmed the diagnosis of LCH. Therapy with intralesional methylprednisolone,¹⁰ as well as oral steroids, chemotherapeutic agents, and radiotherapy¹¹ have been advocated. Surgery and curettage are other treatment modalities.¹² This case showed no signs of spontaneous resolution and deteriorated subsequent to inadvertent incision and drainage. Remarkable regression was seen after 2 intralesional triamcinolone injections.

In conclusion, a tender upper eyelid swelling may be misdiagnosed as lid abscess, leading to exacerbation of the histiocytic lesion at incision. Keeping the age of the patient and the site of involvement in mind, LCH should be considered as a differential diagnosis for such patients.

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