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Abstract
Purpose
Adult xanthogranulomatous disease of the orbit including adult-onset asthma and periocular xanthogranuloma is rare. To the best of the author's knowledge, adult-onset asthma and periocular xanthogranuloma have not been previously reported in Korea.
Case summary
A 75-year-old man presented with a 15-year history of progressive bilateral yellowish eyelid mass. He had a history of adult-onset asthma diagnosed 13 years ago. On ocular examination, there were firm yellowish bilateral eyelid masses and ptosis markedly on the right eye. Exophthalmos was noted in the right eye. There were exotropia, hypertropia, and gaze restriction of the right eye. Orbit magnetic resonance imaging demonstrated an ill-defined bilateral infiltrative orbital mass extending to the lid, lacrimal gland, and sinuses. A right orbital mass extended to the posterior orbit. Enlargement of extraocular muscles was also noted in the right eye. Left upper eyelid mass debulking surgery was performed. A combination therapy of azathioprine and prednisolone was started. Mass debulking surgeries of the right upper and lower eyelids were performed.
References
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Figure 1.
Clinical photographs of patient. (A) Clinical photograph of patient shows bilateral eyelid mass with yellowish pigmentation and severe ptosis more marked in the right eye. (B) Yellowish infiltration of the right temporal and inferior bulbar conjunctiva is noted. (C) Ultra-widefield retinal imaging reveals crystalline lens dislocation into the vitreous cavity of right eye. (D) There are exotropia and hypertropia of right eye on primary gaze and restriction of upward, downward, and medial gaze.
Figure 2.
Orbit magnetic resonance imaging taken 2 years ago demonstrates ill-defined bilateral infiltrative orbital mass extending to lid, lacrimal gland, anterior orbit, nasal cavity, and sinuses. Mass infiltrates posterior orbit and extraocular muscles of right eye. Mass shows low signal intensity on T1-weighted images (T1WI) (A, B), heterogeneous high signal intensity on T2-weighted images (T2WI) (C, D), and strong enhancement by gadolinium contrast medium (T1 c CE) (E, F). Crystalline lens is not dislocated. After 2 years, orbital mass enlarges and infiltrates entire orbit (G-L). Exophthalmos progressed. Right orbital mass extends to orbital apex and enlargement of extraocular muscles is aggravated. Crystalline lens is dislocated into the vitreous cavity.
Figure 3.
Histopathological examination of mass. (A) Histopathology shows granulomatous infiltration consisted of Touton giant cells, xanthoma cells (foamy histiocytes), and inflammatory cells with follicle formation (hematoxylin-eosin, ×100). (B) The section demonstrates Touton giant cells (arrows) and xanthoma cells (arrowhead) (hematoxylin-eosin, ×200).
Figure 4.
Serial preoperative and postoperative photographs of patient. (A) Clinical photograph of the patient before treatment shows bilateral eyelid mass and severe ptosis more marked in the right eye. (B) Left eyelid ptosis is improved two months after levator resection and mass debulking surgery of left upper eyelid. Note decreased tumor infiltration of right eyelid after systemic use of steroid and azathioprine. (C) One month after right upper eyelid surgery, improvement of right eyelid ptosis is noted. (D) Right lower eyelid mass is also improved one year after right lower eyelid surgery. There is marked improvement of eyelid mass with sat-isfactory cosmetic result.
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