Jessner Lymphocytic Infiltration of the Skin

Updated: Dec 13, 2017
  • Author: Kara Melissa T Torres, MD, DPDS; Chief Editor: William D James, MD  more...
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Overview

Background

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.

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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. [6, 7] 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. [8, 9] Data presented by Tomasini et al, [10] 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). 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. [11, 12]

The following four 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. [13]

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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. [14] Another patient presented with a lesion that appeard 3 weeks after starting therapy with etanercept. [15]

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.

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Epidemiology

Frequency

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

Race

Lymphocytic infiltration of the skin has no known racial predilection.

Sex

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.

Age

Lymphocytic infiltration of the skin affects mostly adults younger than 50 years. It has been reported in children. Familial occurrence has been reported. [4]

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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. [16] 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.

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Patient Education

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

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