Extranodal Rosai–Dorfman Disease of the Orbit: Clinical Features of 8 Cases

Tan, Jonathan M.; Narang, Sherry; Purewal, Baljeet; Langer, Paul D.; Blaydon, Sean M.; Schwarcz, Robert; McCormick, Steven A.; Kim, Joon Y.; Walrath, Joseph D.; Burt, Ben; Shinder, Roman

doi:10.1097/iop.0000000000000589

Cited by 17 publications

(21 citation statements)

References 10 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…We conducted a literature search of all cases of RDD involving the lacrimal gland (data summarized in Table 1). Including our patient, there were only 17 cases of RDD involving the lacrimal gland reported in the literature (Table 1 [9,10,16,17,[20][21][22][23][24][25][26][27][28]). The median age of these patients was 32 years (range 4-78).…”

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Atypical Case of Rosai-Dorfman Disease of the Lacrimal Gland with Adjacent Bone Erosion

et al. 2018

View full text Add to dashboard Cite

Background/Aims: Rosai-Dorfman disease (RDD) is a rare, self-limited disorder of unknown etiology that affects children and young adults worldwide and typically manifests as chronic, painless cervical lymphadenopathy. Orbital involvement is very rare and may be an isolated extranodal manifestation or associated with concurrent systemic disease. Adjacent bone involvement is most exceptional, and secondary optic neuropathy has never been reported. Methods: This is a case report with review of the literature. Results: We present a 32-year-old man who, over a 3-month period, developed worsening vision, headache, and vertical diplopia. On examination, there was decreased vision with dyschromatopsia, proptosis, and hypotropia of the left eye. CT scan of the orbits revealed a soft tissue mass inseparable from the lacrimal gland with adjacent bone erosion. Histopathologic evaluation revealed a diffuse infiltrate of histiocytes, lymphocytes, plasma cells, and neutrophils with peripolesis and emperipolesis (tunneling of lymphocytes and plasma cells in the histiocytes’ cytoplasm without destruction), consistent with RDD. Resolution of symptoms as well as of the optic neuropathy was achieved with oral corticosteroids. Conclusion: RDD is an important diagnosis that must be considered in the differential diagnosis of an orbital mass.

show abstract

Section: Discussionmentioning

confidence: 99%

“…Involvement of orbital soft tissue, eyelid, lacrimal gland, conjunctiva, cornea, and retina has been reported. Isolated extranodal manifestations are extremely rare [2,[7][8][9][10].…”

Section: Introductionmentioning

confidence: 99%

Atypical Case of Rosai-Dorfman Disease of the Lacrimal Gland with Adjacent Bone Erosion

et al. 2018

View full text Add to dashboard Cite

show abstract

“…Different types of skin lesions have been described such as infiltrated erythematous/brownish/yellowish papules, nodules, subcutaneous masses, ulcerated nodules, and rosacea‐like lesions affecting most commonly the face but also other parts of the body . Ocular or periorbital lesions including periorbital masses and eyelid lesions have also been reported . Yellowish papules on the eyelids, periorbital area, or rim of the eye concomitant with “xanthelasma‐like” appearance on other parts of the face and extention to the neck, bilateral eyelid swelling, and yellow xanthomatous eyelid masses were some of the reported examples for eyelid lesions.…”

Section: Non‐langerhans Cell Histiocytosesmentioning

confidence: 99%

“…[51][52][53] Ocular or periorbital lesions including periorbital masses and eyelid lesions have also been reported. [53][54][55] Yellowish papules on the eyelids, periorbital area, or rim of the eye concomitant with "xanthelasma-like" appearance on other parts of the face and extention to the neck, 52 bilateral eyelid swelling, 54 and yellow xanthomatous eyelid masses 55 were some of the reported examples for eyelid lesions. Ocular lesions may cause proptosis, blepharoptosis, uveitis, impaired extraocular motility, decreased vision, and diplopia.…”

Section: Reticulohistiocytosismentioning

confidence: 99%

The clinical spectrum of xanthomatous lesions of the eyelids

et al. 2017

View full text Add to dashboard Cite

Yellowish papules, nodules, or plaques, namely "xanthomatous" lesions, may be seen on the eyelids in the course of various disorders. The prototype is "xanthelasma palpebrarum" (XP) that is localized only to the eyelids and may be associated with hyperlipidemia. On the other hand, different types of normolipemic disorders may also cause xanthomatous eyelid lesions. Among these, Langerhans cell histiocytosis, diffuse normolipemic xanthoma, and non-Langerhans cell histiocytoses (papular xanthoma, juvenile xanthogranuloma, xanthoma disseminatum, adult-onset xanthogranuloma, adult-onset asthma and periocular xanthogranuloma, necrobiotic xanthogranuloma, Erdheim-Chester disease, Rosai-Dorfman disease, and reticulohistiocytosis) can be listed. The eyelid findings of this heterogeneous group of disorders are challenging to differentiate from each other due to common clinical aspects that may even sometimes mimic XP. Nodularity, induration, ulceration, diffuse eyelid involvement, and extension from eyelids to the neighboring skin may represent the clinical features of xanthomatous lesions other than XP. It is necessary to obtain a thorough history and exclude XP and then perform detailed dermatological and systemic examination, biopsy for histopathologic confirmation, and appropriate specific imaging screens. As some of the conditions may be associated with other systemic disorders, especially malignancies, the differentiation of xanthomatous eyelid lesions has a critical importance, and clinical signs can be guiding.

show abstract

“…The treatment of choice for RDD confined to the orbit is surgical excision, which, in most cases, leads to disease resolution or stability [233][234][235][236][237]. Regarding nonsurgical treatment, remission has been induced with combinations of systemic steroids and rituximab [238].…”

Section: Rosai-dorfman Diseasementioning

confidence: 99%