Jessner lymphocytic infiltration of the skin (ORPHA:33314)
A rare chronic benign cutaneous disease characterized by asymptomatic non-scaly erythematous papules or plaques on the face and neck.
Epidemiology
Jessner lymphocytic infiltration of the skin (JLIS) is rare, but the exact prevalence is unknown. There is no sex or ethnic predominance. It usually occurs in adults between 30 and 50 years of age. Few cases are reported in childhood.
Clinical description
Cutaneous lesions are mainly localized on the malar region of the face, neck, shoulders, upper back, arms, and hands (sun-exposed skin). Their diameter usually varies between 1 cm and 3 cm. They consist of annular, pink or red papules, sometimes resulting in plaques. No follicular plugging or atrophy appears on the surface of the lesions. The lesions may be singular (approximately 15% of cases) or multiple. When multiple, they are often grouped with an arciform disposition. The lesions may have a chronic waxing and waning course, or more rarely, remain unchanged for several weeks. Onset or exacerbation of the lesions may occur after sun exposure. In general, the lesions are asymptomatic, but they may be sometimes associated with a burning sensation or pruritus. They frequently spontaneously disappear, without resulting scars.
Etiology
JLIS is an inflammatory disease of unknown etiology caused by an accumulation of T cells within the dermis of the skin. There may be a genetic/hereditary component due to multiple reported familial cases. A history of photosensitivity may be associated with the disease. Drug induced JLIS have been described as a reaction to ustekinumab, etanercept, leflunomide, glatiramer acetate, duloxetine and ACE inhibitors. Association with cutaneous B cell lymphoma, Borrelia burgdorferi infection and immune reconstitution syndrome in HIV has been reported.
Diagnostic methods
Diagnosis may be only clinical. Histology reveals nodular perivascular or periadnexal lymphocytes, and dermal infiltration under a normal or only slightly modified epidermis. Infiltrate consists predominantly of reactive CD8+ T cells, with a few CD4+ T cells and B cells. Clusters of plasmacytoid dendritic cells (CD123+) mostly accumulated in the deep vascular plexus, have been described in JLIS as well with identical pattern in lupus erythematous tumidus. Mild mucin deposition may be seen in the dermis. Direct immunofluorescence analyses are usually negative (absence of lupus band test).
Differential diagnosis
Differential diagnoses include rosacea, granuloma annulare, granuloma faciale, cutaneous B-cell lymphomas or pseudolymphoma and idiopathic photodermatoses such as polymorphic light eruption. The differential diagnosis with lupus erythematosus tumidus is much more difficult and some recent studies suggest JLIS cannot be separated from lupus tumidus.
Management and treatment
Treatment of JLIS is not mandatory in all cases. Topical and intralesional corticosteroids are first line therapies. In cases in which photosensitivity is relevant antimalarial drugs (chloroquine and hydroxychloroquine) are generally effective. Multiple therapies have been used even if data on efficacy are scarce, including topical calcineurin inhibitors, auranofin, methotrexate, etretinate, proquazone, thalidomide, and even pulsed dye laser and photodynamic therapy. Photoprotection is advised. Remission, exacerbation or spontaneous resolution may occur.
Prognosis
The overall prognosis is favorable and no systemic involvement has been reported so far.

                            Last update: 
                            June 2024


                            - Expert reviewer(s): 
                            Pr Giampiero GIROLOMONI