Benign lymphoepithelial lesion (BLEL), historically termed Mikulicz’ disease, is a benign inflammatory condition marked by painless enlargement of major exocrine glands, particularly the parotid and lacrimal glands. It is characterized histologically by dense lymphocytic infiltration and associated epithelial proliferation within glandular tissue. BLEL may occur in isolation or in association with autoimmune disorders. It is frequently, but not always, associated with Sjögren’s syndrome. Although BLEL is benign, long-standing lymphoepithelial lesions, particularly in patients with Sjögren’s syndrome, are associated with an increased risk of malignant transformation to extranodal marginal zone B-cell lymphoma (MALT lymphoma).
Signs and symptoms Clinically, a benign lymphoepithelial lesion manifests as:
painless bilateral symmetric swelling of the lacrimal and salivary glands xerostomia (mouth dryness) due to salivary gland dysfunction and xerophthalmia (eye dryness) due to lacrimal gland dysfunction
Treatment Benign lymphoepithelial lesions, particularly when classified as part of IgG4-related disease rather than classic Sjögren’s syndrome, are typically managed with systemic corticosteroids, which often lead to rapid reduction in gland enlargement and improvement in symptoms. Glucocorticoids such as prednisolone or methylprednisolone are commonly used and can result in decreased serum IgG4 levels and shrinkage of affected glands. In cases where steroid response is inadequate or relapse occurs, additional immunosuppressive therapy (e.g., azathioprine, mycophenolate, rituximab) may be considered as steroid-sparing or adjunctive treatment. Supportive care for associated dryness (e.g., artificial tears or saliva substitutes) may also be used when glandular dysfunction is present. Long-term monitoring is important due to the chronic nature of the disease and potential for recurrence.
Eponym Historically, bilateral parotid and lacrimal gland enlargement was characterized by the term Mikulicz' disease if the enlargement appeared apart from other diseases. If it was secondary to another disease, such as tuberculosis, sarcoidosis, lymphoma, and Sjögren's syndrome, the term used was Mikulicz's syndrome. Both names derive from Jan Mikulicz-Radecki, the Polish surgeon best known for describing these conditions. In more recent times, the terms "Mikulicz's disease" and "Mikulicz's syndrome" were viewed as ambiguous and outdated by some sources. Today Mikulicz's disease is considered to be a subtype of IgG4-related disease, usually accompanied by involvement of one or more other organs in the body.
See also Lymphoepithelial lesion
References
Further reading Kahn, LB (Jan 1979). "Benign lymphoepithelial lesion (Mikulicz's disease) of the salivary gland: an ultrastructural study". Human Pathology. 10 (1): 99–104. doi:10.1016/S0046-8177(79)80077-5. PMID 428999. Lee S, Tsirbas A, McCann J, Goldberg R (2006). "Mikulicz's disease: a new perspective and literature review". European Journal of Ophthalmology. 16 (2): 199–203. doi:10.1177/112067210601600201. PMID 16703534. S2CID 24906450. Tsubota, K; Fujita, H; Tsuzaka, K; Takeuchi, T (Jun 2000). "Mikulicz's disease and Sjögren's syndrome". Investigative Ophthalmology & Visual Science. 41 (7): 1666–73. PMID 10845583. Azzopardi, JG; Evans, DJ (Nov 1971). "Malignant lymphoma of parotid associated with Mikulicz disease (benign lymphoepithelial lesion)". Journal of Clinical Pathology. 24 (8): 744–752. doi:10.1136/jcp.24.8.744. PMC 477147. PMID 4943296. Yamamoto, M; Harada, S; Ohara, M; Suzuki, C; Naishiro, Y; Yamamoto, H; Takahashi, H; Imai, K (Feb 2005). "Clinical and pathological differences between Mikulicz's disease and Sjögren's syndrome". Rheumatology. 44 (2): 227–34. doi:10.1093/rheumatology/keh447. PMID 15509627. Delaney, William E.; Balogh, Károly (1966). "Carcinoma of the parotid gland associated with benign lymphoepithelial lesion (Mikulicz's disease) in Sjögren's syndrome". Cancer. 19 (6): 853–860. doi:10.1002/1097-0142(196606)19:6<853::AID-CNCR2820190617>3.0.CO;2-N. PMID 5939056.
External links
Mikulicz syndrome on The National Organization for Rare Disorders (NORD) Benign lymphoepithelial lesions on Radiopedia


