Ocular Adnexal Langerhans Cell Histiocytosis Clinical Features and Management

Abstract

Unifocal, unisystem LCH often presents to the ophthalmologist and can usually be diagnosed on clinical and imaging grounds. Computed tomography and magnetic resonance imaging are complementary imaging studies. Biopsy is essential to confirm diagnosis. Unifocal, unisystem disease can be treated with local excision and curettage. Incomplete excision, recurrent disease or multifocal disease may require systemic chemotherapy.

Langerhans Cell Histiocytosis of the Orbit: Five Clinicopathologic Cases and Review of the Literature

“…18 Caucasians seem to be more frequently affected than other races. 14, 15, 18 However, our case series comprise African-American, Caucasian, and Hispanic patients. Some regard orbital involvement as uncommon, 19, 20 while others reported relatively high incidence rates of orbital LCH (37.5% and 23%).…”

“…17 LCH is more common in boys than girls. 9, 11, 12, 14, 15, 18 LCH in boys is usually diagnosed at a higher age than in girls who present with more severe organ involvement. 18 Caucasians seem to be more frequently affected than other races.…”

“…9, 11, 12, 14, 15, 18 LCH in boys is usually diagnosed at a higher age than in girls who present with more severe organ involvement. 18 Caucasians seem to be more frequently affected than other races. 14, 15, 18 However, our case series comprise African-American, Caucasian, and Hispanic patients.…”

“…Although factors such as infections (such as EBV, CMV, HHV-6), genetic disorders, malignancies (medulloblastoma, retinoblastoma, medullary astrocytoma, glioma), seasonal and environmental influences have been implicated in the pathogenesis of LCH 14, 15, 18, 19, 29, 30 , there is no evidence for one factor to play a pivotal role. 9, 11, 14, 31 However, each single factor may influence the immune system and thus predispose somewhat to LCH.…”

Langerhans Cell Histiocytosis

“…The involved age groups vary by clinical presentation. Multisystem LCH usually occurs before 3 years of age, and unifocal disease is more common in later childhood [ 7 ]. Ocular adnexal LCH often presents between 4 and 8 years old [ 8 ].…”