Diseases title

Jessner's lymphocytic infiltration of the skin )

Orpha Number: 33314
Jessner's benign lymphocytic infiltration of the skin, Jessner-Kanof lymphocytic infiltration of the skin

Jessner lymphocytic infiltration of the skin (JLIS) is a chronic benign cutaneous disease characterized by asymptomatic non-scaly erythematous papules or plaques on the face and neck. The disease is rare, but the exact prevalence is unknown. Onset usually occurs between 30 and 50 years of age. Cutaneous lesions are mainly localized on the back, face, neck, shoulders, arms, and hands (sun-exposed skin). Their diameter usually varies between 2 mm and 2 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 remain unchanged for several months. 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 may spontaneously disappear, without resulting scars. JLIS is an inflammatory disease of unknown etiology caused by an accumulation of T cells within the dermis of the skin. A history of photosensitivity may be associated with the disease. The diagnosis is based on examination of a routine skin biopsy sample, revealing nodular perivascular or periadnexal lymphocytes, and dermal infiltration under a normal or only slightly modified epidermis. Direct immunofluorescence analyses are usually negative (absence of lupus band test). Differential diagnoses include granuloma annulare, granuloma faciale, cutaneous B-cell lymphomas (see this term) and idiopathic photodermatoses such as polymorphic light eruption. The differential diagnosis with lupus erythematosus tumidus (LET, see this term) is much more difficult and some recent studies suggest JLIS cannot be separated from LET. Treatment of JLIS is not mandatory in all cases. Antimalarial drugs (chloroquine and hydroxychloroquine) are generally used asa first line therapy. Thalidomide may also be used, but is strictly contraindicated for pregnant women and women of childbearing age without contraception. Management also includes photoprotection. Remission, exacerbation or spontaneous resolution may occur. The overall prognosis is favorable and no systemic involvement has been reported so far.
source: Orphanet