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Author: Bassam Zeina, MD, PhD, Consulting Staff, Department of Dermatology, Milton Keynes Hospital, United Kingdom

Coauthor(s): Mohsin Ali, MBBS, MRCP, MRCPI, Consulting Staff, Department of Dermatology, Amersham General Hospital, UK; Sohail Mansoor, MBBS, MSc, Dermatologist and Lead Physician in Dermatologic Surgery, Department of Dermatology, Barnet Hospital, London, UK

Editors: Peter Fritsch, MD, Chair, Department of Dermatology and Venereology, University of Innsbruck, Austria; Richard P Vinson, MD, Assistant Clinical Professor, Department of Dermatology, Texas Tech University School of Medicine; Consulting Staff, Mountain View Dermatology, PA; Daniel S Loo, MD, Associate Professor, Residency Program Director, Department of Dermatology, Boston University School of Medicine; Glen H Crawford, MD, Assistant Clinical Professor, Department of Dermatology, University of Pennsylvania School of Medicine; Chief, Division of Dermatology, The Pennsylvania Hospital; William D James, MD, Paul R Gross Professor of Dermatology, University of Pennsylvania School of Medicine; Vice-Chair, Program Director, Department of Dermatology, University of Pennsylvania Health System

Author and Editor Disclosure

Synonyms and related keywords: Jessner's lymphocytic infiltrate, benign chronic T-cell infiltrative disorder, Jessner LIS, Jessner's LIS, lupus erythematosus tumidus, LET, discoid lupus erythematosus, DLE, broad-spectrum photosensitivity disorder

Background

Jessner and Kanof1 first described this uncommon condition in 1953. The condition now known as Jessner lymphocytic infiltration of the skin (LIS) 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. Older literature would suggest that this is not correct and that certain patients monitored for as long as 30 years remain within the spectrum of LIS with no progression. However, more recent literature suggests that LIS cannot be separated from lupus erythematosus tumidus (LET) clinically, histologically, or photobiologically.2

Pathophysiology

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

Frequency

United States

The frequency of this condition in the United States is unknown.

International

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

Mortality/Morbidity

LIS is not associated with increased mortality. The lesions are commonly asymptomatic, although some patients report burning or pruritus.

Race

LIS 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

LIS affects mostly adults younger than 50 years. It has been reported in children. Familial occurrence has been reported.



History

Patients with Jessner lymphocytic infiltrate commonly present with asymptomatic, nonscaly, erythematous papules or plaques on the face and neck of several months duration.

Onset or exacerbation of lesions following sun exposure may or may not be noted. Some patients report burning or pruritus, and rarely, a familial occurrence has been noted. The course of LIS 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 LIS over polymorphous light eruption.

Physical

Pertinent physical findings are limited to the skin.

  • Primary lesion
    • LIS usually begins as nonscaly erythematous papules, which expand peripherally, forming well-demarcated, slightly infiltrated red 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
    • The malar area of the face and the upper back are the most common sites of involvement.
    •  Other parts of the body may be affected as well, including the forehead, neck, mastoid region, arms, legs, chest, and abdomen.

Causes

The cause of LIS is unknown. Over the years, a number of etiologies have been proposed. Several studies have linked Borrelia infection with LIS, yet most recent studies have disputed this association. Recent 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.



Granuloma Annulare
Granuloma Faciale

Other Problems to be Considered

Lupus erythematosus tumidus
Gyrate erythema



Lab Studies

Serologic testing for 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.

Other Tests

  • Phototesting: Both UVA and/or UVB elicit abnormal papular phototest reactions resembling lesions of LIS 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 LIS should be sought and confirmed using provocative phototesting. This relevant event could guide the therapeutic strategy because antimalarials have been effective for Jessner LIS 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

LIS 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 LIS, are often referred to as the 5 L's.

The following histologic findings are characteristic of LIS, 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.

Eosinophils and neutrophils are lacking, and their presence should cause one to suspect an infectious etiology or arthropod bite reaction, rather than LIS.

Copious dermal mucin is not observed, which helps distinguish LIS 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.

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, HLA-DR–negative T cells compared to discoid lupus may help distinguish those conditions.

The presence of natural killer (NK) cells in Jessner lymphocytic infiltrate has been disputed. More reactive B cells may be found in the infiltrate than in DLE.



Medical Care

Prognosis is good because LIS may resolve spontaneously. It may require no treatment. Treatments include cosmetic camouflage, photoprotection, excision of small lesions, topical steroids, oral hydroxychloroquine, intralesional steroids, systemic steroids, and cryotherapy.

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.



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

Drug Category: Corticosteroids

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.

Drug NameHydrocortisone valerate (Westcort)
DescriptionAnti-inflammatory adrenocorticosteroid derivative suitable for application to skin or external mucous membranes. May alleviate pruritus and speed resolution of lesions. Available as a 0.2% cream or ointment.
Adult DoseApply sparingly to affected areas bid
Pediatric DoseNot established
ContraindicationsDocumented hypersensitivity; viral, fungal, and bacterial skin infections
InteractionsNone reported
PregnancyC - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
PrecautionsProlonged use over large surface areas and/or use of occlusive dressings may increase systemic absorption of corticosteroids and may cause Cushing syndrome, reversible HPA axis suppression, hyperglycemia, and glycosuria; do not use on eyelids for >2 wk

Drug NameTriamcinolone (Aristocort ointment, Kenalog cream)
DescriptionFor inflammatory dermatosis responsive to steroids; decreases inflammation. Intramuscular injection may be used for widespread skin disorders, or intralesional injections may be used for localized skin disorders.
Adult Dose2.5-10 mg/mL intralesionally; may be given IM in a concentration of 40 mg/mL; also comes as a 0.1% cream and ointment
Pediatric Dose<12 years: Not established
>12 years: Administer as in adults
ContraindicationsDocumented hypersensitivity; fungal, viral, and bacterial skin infections
InteractionsCoadministration with barbiturates, phenytoin, and rifampin decreases effects of triamcinolone
PregnancyC - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
PrecautionsIf given IM, bruising, infection, cold abscess, temporary skin depression, and/or temporary skin discoloration (usually lightened) may occur at the injection site; psychiatric effects such as being "wired" or "weird" may occur and may disrupt sleep; increased susceptibility to infections; increased blood glucose levels; peptic ulcer disease; steroid withdrawal syndrome; many other adverse effects possible and prescribing physician should be familiar with these

Drug NameBetamethasone dipropionate (Diprosone lotion)
DescriptionFor inflammatory dermatosis responsive to steroids; decreases inflammation. Available as 0.05% cream and ointment.
Adult DoseApply thin film bid until response
Pediatric Dose<12 years: Not recommended
ContraindicationsDocumented hypersensitivity; viral, fungal, and bacterial skin infections
InteractionsNone reported
PregnancyC - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
PrecautionsCan cause atrophy of groin, face, and axillae or striae distensae; rosacealike eruption on discontinuation of use; may increase skin fragility; may suppress HPA axis if used over large surface area over a long period; tachyphylaxis may occur if used >2 wk

Drug NamePrednisone (Deltasone, Orasone)
DescriptionFor treating LIS, when taken PO, is used alone or in combination with topical or intralesional steroids. May decrease inflammation by reversing increased capillary permeability and suppressing PMN activity.
Adult Dose1-1.5 mg/kg/d initial qam or in divided doses; titrate dose to clinical response; taper PO steroids when disease is inactive
Pediatric DoseAdminister as in adults
ContraindicationsDocumented hypersensitivity; viral infection; peptic ulcer disease; hepatic dysfunction; connective tissue infections; fungal or tubercular skin infections; GI disease
Relative contraindications include hypertension, active tuberculosis, congestive heart failure, prior psychosis, positive IPPD test result, glaucoma, severe depression, diabetes mellitus, active peptic ulcer disease, cataracts, osteoporosis, recent bowel anastomosis, and pregnancy
InteractionsCoadministration with estrogens may decrease clearance; when used with digoxin, digitalis toxicity secondary to hypokalemia may increase; phenobarbital, phenytoin, and rifampin may increase the metabolism of glucocorticoids (consider increasing maintenance dose); monitor for hypokalemia with coadministration of diuretics
PregnancyB - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
PrecautionsAbrupt discontinuation may cause adrenal crisis; hyperglycemia, edema, osteonecrosis, myopathy, peptic ulcer disease, hypokalemia, osteoporosis, euphoria, psychosis, myasthenia gravis, growth suppression, and infections may occur with glucocorticoid use; risk of cataracts, osteonecrosis, and osteoporosis are not minimized by alternate-day therapy; HPA axis advantages of qod dosing disappear once physiologic levels (10-15 mg prednisone qod) are met

Drug Category: Antimalarials

Used to treat certain photosensitive eruptions including LIS.

Drug NameHydroxychloroquine (Plaquenil)
DescriptionMechanism of action not fully understood. Postulates include light filtration, immunosuppression, anti-inflammatory, and DNA binding. Supplied as 200-mg tabs.
Adult Dose100-200 mg PO bid
Pediatric Dose10 mg base/kg/d PO qd or divided bid initially, followed by 5 mg/kg at 6, 24, and 48 h
ContraindicationsDocumented hypersensitivity; retinopathy from any cause
Relative contraindications include pregnancy/lactation, retinal/visual-field changes, severe blood dyscrasias, psoriasis, G-6-PD deficiency, significant hepatic dysfunction, myasthenia gravis, significant neurologic disease, long-term therapy in children
InteractionsCimetidine may increase levels; kaolin and magnesium trisilicate may decrease levels may increase digoxin levels; increased retinal toxicity with chloroquine and hydroxychloroquine (do not give together)
PregnancyC - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
PrecautionsCaution in hepatic disease, G-6-PD deficiency, psoriasis, and porphyria; not recommended for long-term use in children (main concern is overdose/toxicity; chronic toxicity risk, however, is felt to be no greater than in adults); perform periodic (baseline and yearly) ophthalmologic examinations; test periodically for muscle weakness; check blood cell counts periodically; hemolysis, agranulocytosis, aplastic anemia, and leukopenia can occur



Further Inpatient Care

  • Inpatient care is not generally required.

Further Outpatient Care

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

Deterrence/Prevention

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

Prognosis

  • Prognosis is good because LIS may resolve spontaneously.

Patient Education

  • Provide education relating to the benign nature of LIS.



Medical/Legal Pitfalls

  • Failing to exclude other conditions that may appear clinically similar to LIS and demonstrate a patchy lymphocytic infiltrate histologically, such as lupus erythematosus or lymphocytic lymphoma: When in doubt, obtain a second biopsy or perform additional investigations as indicated.
  • Failure to reexamine patients before repeatedly refilling patients' steroids: Steroid side effects may progress to the point of permanent damage without appropriate surveillance.
  • Failure to consider a new problem when a patient who previously was doing well suddenly seems to worsen: Allergic contact dermatitis may develop with any topical therapy (including steroids); irritant dermatitis may likewise develop.



Media file 1:  Jessner lymphocytic infiltration of the skin.
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Media file 2:  Jessner lymphocytic infiltration of the skin.
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Jessner Lymphocytic Infiltration of the Skin excerpt

Article Last Updated: Oct 17, 2007