Nonosseous Periocular Manifestations of Langerhans Cell Histiocytosis: A Case Report and Systematic Review

Abstract

Purpose: Langerhans cell histiocytosis (LCH) is a disorder of dendritic cell proliferation that typically involves bone. It can be diagnostically challenging when LCH presents without bony involvement, leading to delays in diagnosis and treatment. In this study, the periocular manifestations of LCH in cases where the underlying orbital bones are not involved are described through a systematic review. Methods: A systematic review of the literature was performed to capture all cases of LCH that involved the periocular region but not the underlying orbital bones. These included LCH cases that involved the periocular skin, the ocular surface, and the orbital tissue. The authors also highlight an additional case where LCH presented with periocular edema and multifocal, nodular conjunctival lesions. Result: This review illustrates that LCH rarely presents with periocular infiltration without orbital bone involvement. In these atypical cases, LCH can present as an eyelid mass, a chalazion-like lesion, generalized periocular swelling, ocular surface lesions, or infiltration of any orbital structure. Ocular surface LCH has a higher rate of recurrence than other periocular LCH. Orbital LCH can involve any tissue including extraocular muscles, the lacrimal gland, or indistinct areas within the orbit. Conclusions: LCH is a clinicopathologic diagnosis. Although most cases involve the bone, any soft tissue can be involved. Biopsy is required to confirm the diagnosis of this heterogeneous disease.

Successful treatment of orbital Langerhans cell histiocytosis with stereotactic radiosurgery: A case report and literature review

“…Literature review in PubMed yielded 68 results using the terms “langerhans cell histiocytosis and orbit” of which there are 15 applicable results outlined below. There were no complications with treatment 1,9–22 …”

“…There were no complications with treatment. 1 , 9 , 10 , 11 , 12 , 13 , 14 , 15 , 16 , 17 , 18 , 19 , 20 , 21 , 22 Abbreviations: FNA, fine needle aspiration; ILS, intralesional steroids; IVCT, IV chemotherapy (etoposide, cisplatin), steroids (methylprednisolone or prednisolone); OCT, oral chemotherapy (6 mercaptopurine, vincristine, vinblastine). …”

“…There were no complications with treatment. 1 , 9 , 10 , 11 , 12 , 13 , 14 , 15 , 16 , 17 , 18 , 19 , 20 , 21 , 22 …”