Jessner Lymphocytic Infiltration of the Skin

Updated: Jun 27, 2022
  • Author: Kara Melissa Torres Culala, MD; Chief Editor: William D James, MD  more...
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Overview

Practice Essentials

Jessner lymphocytic infiltrate of the skin (LIS) is a benign yet chronic, T-cell infiltrative disease, first described in 1953 by Jessner and Kanof. [1, 2]  The condition has remained poorly understood, and indeed, the very existence of such a condition has been questioned. One argument is that patients with this condition are simply displaying the early manifestations of some other disorder. Some authors consider this entity to be a variant of lupus erythematosus, [3]  and some consider this to be a CD8+ polyclonal reactive pseudolymphoma. [4, 5]

It usually presents with recurrent, asymptomatic, erythematous and annular papules and plaques with a predilection for sun-exposed sites (see Physical Examination). The clinical course varies with remissions and exacerbations. The lesions may persist indefinitely, and some may disappear spontaneously without sequelae.

Etiology

The cause of Jessner lymphocytic infiltration of the skin (LIS) is unknown. Over the years, a number of etiologies have been proposed.

One study has linked Borrelia infection with lymphocytic infiltration of the skin. [5]  However, Borrelia infection may present with similar histology findings and can represent a pseudolymphomatous reaction (Borrelia -associated pseudolymphoma) and should be excluded, especially in countries with high rates of Borrelia infections. [5]

One occurrence of LIS as a consequence of Koebner response to patch testing has been reported. [6]  Another patient presented with a lesion that appeard 3 weeks after starting therapy with etanercept. [7]  There has been a single reported case of LIS as a response to hirudotherapy. [8]

Other data suggest that a history of photosensitivity in patients with Jessner LIS should be sought and confirmed using provocative phototesting. In cases in which photosensitivity is relevant, antimalarials are expected to be effective.

Diagnostics

Serologic testing for systemic lupus erythematosus (SLE) should be considered. This testing should include, but is not limited to, screening antinuclear antibodies (ANA), erythrocyte sedimentation rate (ESR), anti-Ro (Sjögren syndrome A [SSA]) and anti-La (Sjögren syndrome B [SSB]) antibodies, CBC count, and urinalysis. [9]

Immunophenotyping may be valuable in differentiation from cutaneous lymphoma (polyclonal vs monoclonal).

Other tests

Both UVA and/or UVB elicit abnormal papular phototest reactions resembling lesions of lymphocytic infiltration of the skin both clinically and histologically in some patients. Lesions typically develop 3-6 days after the first UV exposure. Some data suggest that a photosensitivity history in patients with Jessner lymphocytic infiltration of the skin should be sought and confirmed using provocative phototesting. This relevant event could guide the therapeutic strategy because antimalarials have been effective for Jessner lymphocytic infiltration of the skin patients with photosensitivity.

Procedures

Skin biopsy is the primary diagnostic study. Biopsy from a relatively new lesion is recommended, and it should be sent for routine hematoxylin and eosin (H&E) staining. Select lesions that have not been treated with topical steroids or other immunosuppressive agents.

A portion of the specimen, or an additional biopsy, may be immediately frozen in liquid nitrogen and stored at –70°C for immunohistochemical studies requiring fresh frozen tissue if later indicated. A biopsy, or portion thereof, should be placed in immunofluorescent transport medium for direct immunofluorescence studies.

Also see Histologic Findings.

Treatment

Also see Medical Care.

Excision of solitary small lesions may be possible.

Consultations

A dermatologist may be consulted to suggest options for cosmetic camouflage.

Prevention

Prevention is not possible because the etiology of lymphocytic infiltration of the skin is unknown. Sun avoidance and photoprotection are strongly recommended in all cases with or without a history of photo-aggravation because lymphocytic infiltration of the skin is very likely a photosensitive disorder.

Long-term monitoring

Regular follow-up is required to monitor for the occurrence of steroid atrophy if potent topical steroids are used.

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Pathophysiology

Whether Jessner lymphocytic infiltrate of the skin (LIS) constitutes a separate disease entity and to what extent it is related to other benign cutaneous lymphocytic infiltrates is not entirely clear.

Some authors propose that LIS may be a dermal variant of lupus erythematosus, polymorphic light reaction, cutaneous lymphoid hyperplasia, or reticular erythematous mucinosis. [10, 11] Evidence links some cases of LIS to Borrelia burgdorferi. [5]

The literature suggests that LIS has many similarities with lupus erythematosus tumidus (LET) clinically, histologically, and photobiologically and can be considered a continuous spectrum. [12, 13] Data presented by Tomasini et al, [14] showed that the plasmacytoid dendritic cells in tumid lupus erythematosus and LIS both present as distinct patterns compared with other forms of dermatitis (see Other tests under Practice Essentials). This finding supports the belief that the LIS is a clinical variant of lupus erythematosus. Another retrospective study, [2] for 10 years with a 4-year follow-up, revealed a high occurrence of typical clinical features of lupus erythematosus, suggesting that LIS may be a cutaneous marker of a subset of lupus erythematosus patients with an excellent prognosis.

Lastly, rare cases of angiotensin-converting enzyme inhibitor– and glatiramer acetate–induced LIS have been described. [15, 16]

The following 4 views have been expressed:

  • It represents an entirely separate entity.

  • Although some cases represent a separate entity, other reported cases are discoid lupus erythematosus (DLE).

  • All cases are DLE or LET, which is a subtype of DLE.

  • It represents an initial phase or abortive stage of any of the other diseases with a patchy dermal lymphocytic infiltrate.

LIS can be viewed as a broad-spectrum photosensitivity disorder, which may demonstrate a delayed provocative phototest reaction. The relationship to sun exposure, consequently, is not always noted by the patient. [17]

Prognosis

Prognosis is good because lymphocytic infiltration of the skin may resolve spontaneously.

Lymphocytic infiltration of the skin (LIS) is not associated with increased mortality. The lesions are commonly asymptomatic, although some patients report burning or pruritus.

LIS has been reported to affect the periorbital skin, resulting to severe cicatricial ectropion. [18]  Observation and follow-up may be prudent, particularly in elderly patients if the lesions were noted to occur near the orbital areas as the condition may be mistaken for an age-related ectropion.

Patient education

Provide education relating to the benign nature of lymphocytic infiltration of the skin.

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Epidemiology

The incidence and prevalence is unknown. It is considered uncommon.

Lymphocytic infiltration of the skin has no known racial predilection. The reported sex ratio varies depending upon the source consulted. Some have reported a male-to-female ratio as high as 10:1, while others have noted a slight female predominance.

Lymphocytic infiltration of the skin affects mostly adults between 30 and 50 years of age. [19] It has been reported in children. [20] Familial occurrence has been reported. [4]

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