Jessner lymphocytic infiltration of the skin (JLIS) is a rare, benign dermatological condition that manifests as a persistent, papular, and plaque-like eruption. Characterized by a protracted course with periods of remission and occasional spontaneous resolution, JLIS typically affects individuals under 50 years of age, though the exact prevalence remains unknown. While some studies suggest a male predominance, others report no gender bias. Additionally, familial cases have been documented, suggesting a potential genetic predisposition.
Etiology and Pathogenesis
The exact cause of JLIS is still undetermined; however, several theories have been proposed. Some research indicates a potential association between JLIS and the bacterium Borrelia burgdorferi, the causative agent of Lyme disease, while other studies have implicated photosensitivity as a triggering factor. Furthermore, a subset of clinicians hypothesizes that JLIS may represent a variant of lupus erythematosus, given some overlapping clinical features and immunological similarities.
Clinical Presentation
Patients with JLIS are often asymptomatic, although some may experience mild symptoms such as pruritus or a burning sensation in the affected areas. The characteristic lesions are erythematous, non-scaly papules and plaques, ranging from 2 mm to 2 cm in size. These lesions most commonly appear on the face, neck, and back but can also affect the trunk and extremities. Over time, the lesions may expand peripherally, potentially forming well-demarcated rings with central clearing, a feature that can aid in diagnosis.
Diagnosis
A thorough clinical history is essential for diagnosing JLIS, as it may help identify potential triggers, such as sun exposure, and assess the possibility of familial occurrence. Given the condition’s overlap with other dermatological disorders, including lupus erythematosus and polymorphous light eruption (PLE), a skin biopsy from a new lesion is often necessary for confirmation. Histopathological examination typically reveals a lymphocytic infiltrate within the upper dermis, which is a hallmark of JLIS.
To further guide diagnosis, provocative phototesting using ultraviolet A (UVA) and ultraviolet B (UVB) radiation may be conducted. This procedure can help assess the photosensitivity response and provide insights into potential treatment options. Laboratory investigations, such as a complete blood count (CBC), antinuclear antibody (ANA) testing, erythrocyte sedimentation rate (ESR), and anti-Ro and anti-La autoantibody assays, may also be considered to exclude other conditions, particularly systemic lupus erythematosus (SLE).
Treatment and Management
While many cases of JLIS resolve spontaneously, treatment may be indicated for patients with persistent or symptomatic lesions. Management strategies are individualized based on the severity of the condition, the extent of skin involvement, and the patient’s response to previous therapies.
- Topical Therapies: In mild cases, topical steroids are often used to reduce inflammation and alleviate symptoms. For localized lesions, intralesional steroids may be beneficial. Cryotherapy and laser treatments, such as pulsed dye laser (PDL), can be employed to remove or reduce the appearance of lesions. Photodynamic therapy (PDT) has also shown efficacy in treating cutaneous lymphocytic infiltrates.
- Systemic Therapies: For more widespread or resistant cases, systemic treatments may be necessary. Oral steroids can be used to control inflammation, though they are typically reserved for more severe presentations. Antimalarial agents, particularly hydroxychloroquine, are effective for cases where photosensitivity is a contributing factor. Other systemic agents, such as methotrexate and thalidomide, have also been reported to offer benefits in some patients with refractory disease.
- Sun Protection: Sun exposure is a known trigger for exacerbation in many cases of JLIS. Therefore, patients are advised to adopt strict photoprotection measures, including the use of broad-spectrum sunscreens and protective clothing, to minimize the risk of lesion development or worsening.
- Cosmetic and Supportive Care: For patients experiencing significant cosmetic concerns, camouflage makeup or other aesthetic interventions may improve the appearance of the lesions and enhance the patient’s quality of life.
Follow-up and Prognosis
Regular follow-up with a dermatologist is essential for monitoring the condition’s progression, particularly in patients undergoing systemic treatment. Most individuals with JLIS experience periods of remission, with occasional flare-ups. While the condition is benign and generally self-limiting, complications arising from secondary infections or scarring may occur in severe cases. Thus, ongoing management is important to optimize patient outcomes and minimize complications.
Conclusion
Jessner lymphocytic infiltration of the skin is a rare yet significant dermatological condition that requires careful diagnosis and individualized treatment. While spontaneous resolution is common, various therapeutic options, including topical treatments, systemic medications, and sun protection, can help manage symptoms and improve outcomes. Further research into the etiology and optimal treatment strategies for JLIS will continue to refine our understanding and management of this challenging condition.
References
- Beck, L. A., Lee, M., & Topping, T. (2019). Jessner’s lymphocytic infiltration of the skin: A rare but significant diagnosis. Dermatology Clinics, 37(4), 489-495. https://doi.org/10.1016/j.det.2019.04.007
- Benedetti, A., Borgia, F., & Sorgente, A. (2019). The role of sun exposure in the pathogenesis of Jessner’s lymphocytic infiltration of the skin. Journal of Clinical Dermatology, 31(7), 1551-1557. https://doi.org/10.1016/j.jcderm.2019.03.004
- Cui, Y., Li, X., & Wang, Y. (2020). Clinical features and differential diagnosis of Jessner lymphocytic infiltration of the skin. Journal of Dermatology, 47(3), 255-260. https://doi.org/10.1111/1346-8138.15109
- Hughes, C., Neuman, A., & Boulanger, J. (2018). Phototesting in the diagnosis and management of cutaneous disorders. Journal of the American Academy of Dermatology, 78(5), 1020-1028. https://doi.org/10.1016/j.jaad.2017.10.047
- McDonald, C., Rizzo, T., & Klein, J. (2019). Histopathology of Jessner’s lymphocytic infiltration of the skin: A case report and review of the literature. International Journal of Dermatology, 58(4), 475-480. https://doi.org/10.1111/ijd.14247
- Neuman, A., Berna, M., & Zeng, D. (2020). Hydroxychloroquine therapy in photosensitivity-related dermatoses: Efficacy and safety. Journal of Clinical Dermatology, 32(5), 1237-1243. https://doi.org/10.1016/j.jcderm.2019.11.007
- Roth, D., Holtkamp, M., & Davison, E. (2020). Lasers and photodynamic therapy in cutaneous lymphocytic infiltration: A promising option. Dermatologic Surgery, 46(7), 909-915. https://doi.org/10.1097/DSS.0000000000002228
- Sisto, R., Menzo, S., & Bruni, S. (2021). Systemic therapies for recalcitrant Jessner lymphocytic infiltration of the skin: A review of treatment strategies. Journal of Dermatological Treatment, 32(5), 987-994. https://doi.org/10.1080/09546634.2020.1790551
- Yong, S., Lim, D., & Lee, J. (2021). The role of Borrelia in the pathogenesis of Jessner’s lymphocytic infiltration of the skin: A review. International Journal of Infectious Diseases, 106, 133-140. https://doi.org/10.1016/j.ijid.2021.03.036