Jessner Lymphocytic Infiltration of the Skin 

Updated: Dec 13, 2017
Author: Kara Melissa Torres Culala, MD; Chief Editor: William D James, MD 

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.

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]

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.

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]

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.

Patient Education

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

 

Presentation

History

Patients with Jessner lymphocytic infiltrate commonly present with asymptomatic, nonscaly, erythematous papules or plaques predominantly on the face and neck, upper back, or trunk of several months' duration.[17] The lesions may be solitary or numerous. There are no known systemic manifestations.

Onset or exacerbation of lesions following sun exposure may or may not be noted. The lesions have also been observed to reappear at the original site or in previously unaffected areas. Some patients report burning or pruritus, and rarely, a familial occurrence has been noted.[4] The course of lymphocytic infiltration of the skin is variable and unpredictable, most often lasting months to years. Periods of remission and exacerbation may be observed, as well as spontaneous resolution. A history of active lesions involving covered skin or occurrence during winter months can be a helpful clue favoring a diagnosis of lymphocytic infiltration of the skin over polymorphous light eruption.

Physical Examination

Pertinent physical findings are limited to the skin. Note the images below.

Jessner lymphocytic infiltration of the skin. Jessner lymphocytic infiltration of the skin.
Jessner lymphocytic infiltration of the skin. Jessner lymphocytic infiltration of the skin.

Primary lesions are as follows:

  • Lymphocytic infiltration of the skin usually begins as nonscaly erythematous papules, which expand peripherally, forming well-demarcated, slightly infiltrated erythematous plaques.

  • Central clearing may occur.

  • The surface of the lesions appears normal and, in particular, shows no follicular plugging or atrophy.

  • The size of papules or plaques varies from 2 mm to 2 cm in diameter, and they may be arranged in crescents or rings.

  • One, a few, or numerous lesions may be present.

  • After persisting for several months or several years, lesions usually disappear without sequelae, but they may recur at either the same sites or elsewhere.

Distribution is as follows:

  • The malar area of the face and the upper back are the most common sites of involvement.[18]

  • Other parts of the body may be affected as well, including the forehead, neck, mastoid region, arms, legs, chest, and abdomen.

 

DDx

Differential Diagnoses

  • Cutaneous lymphoid hyperplasia

  • Cutaneous lymphoma

  • Granuloma Annulare

  • Granuloma Faciale

  • Gyrate erythema

  • Lupus erythematosus tumidus

  • Plaque form of polymorphous light reaction

  • Reticular erythematous mucinosis

 

Workup

Laboratory Studies

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.[19]

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.

Histologic Findings

Lymphocytic infiltration of the skin is one of a group of conditions characterized histologically by a lymphocytic infiltrate in the dermis. The following are included in this group:

  • Polymorphous light eruption

  • Discoid lupus erythematosus

  • Well-differentiated lymphocytic lymphoma

  • Lymphocytoma cutis

The above conditions, along with Jessner lymphocytic infiltration of the skin, are often referred to as the 5 L 's.

The following histologic findings are characteristic of lymphocytic infiltration of the skin, and helpful differentiating features from other conditions characterized by a lymphocytic infiltrate are noted.

The epidermis usually is normal with no evidence of atrophy, follicular plugging, basal vacuolar change, or basement membrane zone thickening. Those features are more consistent with a diagnosis of DLE.

In the dermis, a moderately dense superficial and deep perivascular lymphocytic infiltrate is observed at scanning magnification. The lymphocytic infiltrate may also be perifollicular or may extend into the subcutis.

Higher magnification reveals a predominance of small mature lymphocytes. Large lymphoid cells, plasma cells, or plasmacytoid monocytes may occasionally be present. Plasmacytoid dendritic cells were also observed as perivascular and periadnexal clusters in both lupus erythematosus and LIS as compared with other forms of dermatitis, which exhibited plasmacytoid dendritic cells as single cells or scattered cells throughout the infiltrate.[10]

Eosinophils and neutrophils are lacking, and their presence should cause one to suspect an infectious etiology or arthropod bite reaction, rather than lymphocytic infiltration of the skin.

Copious dermal mucin is not observed, which helps distinguish lymphocytic infiltration of the skin from tumid lupus erythematous and reticular erythematous mucinosis.

Prominent papillary dermal edema is likewise absent, and its presence would suggest polymorphous light eruption or pernio.

Germinal follicle formation, as would be observed in lymphocytoma cutis or cutaneous follicular center cell lymphoma, is absent, as is a clonal population of B or T cells on molecular analysis.

Findings on direct immunofluorescence are negative, as opposed to classic DLE, which usually has positive findings. Tumid lupus, however, is also usually negative.

One study demonstrated an increase in circulating immune complexes in both reticular erythematous mucinosis and LIS.[7] The same study demonstrated a decrease in the immune complexes with hydroxychloroquine therapy and clinical clearing, with a resurgence upon discontinuation of treatment.

Marker studies reveal that the overwhelming population of infiltrating cells is one of mature T cells. An increased number of Leu-8 (CD62 L)–positive T cells,[20] nonexpression of ICAM-1, and negative epidermal expression of 27E10[21] compared with discoid lupus may help distinguish those conditions. In contrast to earlier studies, an absence of T cells expressing HLA-DR antigens was observed in keratinocytes in lupus erythematosus and LIS.[21] There is also absent HLA-DR expression in polymorphous light reaction.[22]

Natural killer cell function has been found to be decreased in both reticular erythematous mucinosis and LIS.[7]

More reactive B cells may be found in the infiltrate than in DLE.[23]

Lymphocytic infiltrate contains mainly of CD8+ T cells.[17]

 

Treatment

Medical Care

Prognosis is good because lymphocytic infiltration of the skin may resolve spontaneously. It may require no treatment, but some patients benefit from cosmetic camouflage, photoprotection, excision of small lesions, topical steroids, intralesional steroids, oral hydroxychloroquine, systemic steroids, cryotherapy, methotrexate,[24] thalidomide,[25] and/or oral auranofin.[26, 27, 28]

In 2009, Borges da Costa et al treated one patient with a pulsed-dye laser using a 10-mm spot, a fluence of 7 J/cm2, and a single pulse of 0.5 msg. Complete clearing of all lesions was observed after only one treatment, without any adverse effects.[29] The value of pulsed dye laser as a possible first-line treatment for LIS was again analysed in 2010.[30] In 3 of 5 patients, a single treatment session with 6-8 J/cm2 showed complete clearing of the lesions. There was no recurrence in the 3 patients at 4-8 years of follow-up.

Park et al reported a case of a patient with lymphocytic infiltration of the skin, refractory to usual treatments, who was treated with photodynamic therapy (PDT). Lesions showed marked improvement after 2 sessions of methyl 5-aminolevulinic acid PDT.[31] Another option for refractory disease is oral methotrexate at 15 mg/wk for 4 months.[24] There was no recurrence after 2 years of observation.

Surgical Care

Excision of solitary small lesions may be possible.

Consultations

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

Diet

No dietary recommendations are currently proposed.

Activity

No specific activity limits or exercises are recommended. Photoprotection is needed for all patients.

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.

 

Medication

Medication Summary

The goals of pharmacotherapy are to reduce morbidity and prevent complications.

Corticosteroids

Class Summary

These agents have anti-inflammatory properties and cause profound and varied metabolic effects. They modify the body's immune response to diverse stimuli. Oral corticosteroids should only be used in severe unremitting disease not responsive to other treatment.

Hydrocortisone valerate (Westcort)

Hydrocortisone valerate is an anti-inflammatory adrenocorticosteroid derivative suitable for application to skin or external mucous membranes. It may alleviate pruritus and speed the resolution of lesions. Hydrocortisone valerate is available as a 0.2% cream or ointment.

Triamcinolone topical (Aristocort ointment, Kenalog cream)

Topical triamcinolone is for inflammatory dermatoses responsive to steroids; it decreases inflammation. An intramuscular injection may be used for widespread skin disorders, or intralesional injections may be used for localized skin disorders.

Betamethasone topical (Diprosone lotion)

Topical betamethasone is for inflammatory dermatoses responsive to steroids; it decreases inflammation. Betamethasone is available as a 0.05% cream or ointment.

Prednisone (Deltasone, Orasone)

Prednisone is used for treating lymphocytic infiltration of the skin, when taken orally, and is used alone or in combination with topical or intralesional steroids. It may decrease inflammation by reversing increased capillary permeability and suppressing PMN activity.

Antimalarials

Class Summary

Antimalarials are used to treat certain photosensitive eruptions, including lymphocytic infiltration of the skin.

Hydroxychloroquine (Plaquenil)

Hydroxychloroquine's mechanism of action is not fully understood. Theories include light filtration, immunosuppression, anti-inflammatory action, and DNA binding. It is supplied as 200-mg tablets.