eMedicine Specialties > Dermatology > Lymphoma and Related Processes
Jessner Lymphocytic Infiltration of the Skin
Updated: Dec 31, 2008
Introduction
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 lymphocytic infiltration of the skin with no progression. However, more recent literature suggests that lymphocytic infiltration of the skin 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.
Lymphocytic infiltration of the skin 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
Lymphocytic infiltration of the skin is not associated with increased mortality. The lesions are commonly asymptomatic, although some patients report burning or pruritus.
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.
Clinical
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.3 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
Pertinent physical findings are limited to the skin.
- Primary lesion
- Lymphocytic infiltration of the skin 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 lymphocytic infiltration of the skin is unknown. Over the years, a number of etiologies have been proposed. Several studies have linked Borrelia infection with lymphocytic infiltration of the skin, yet most more recent studies have disputed this association. Other data suggest that a history of photosensitivity in patients with Jessner lymphocytic infiltration of the skin should be sought and confirmed using provocative phototesting. In cases in which photosensitivity is relevant, antimalarials are expected to be effective.
Differential Diagnoses
Granuloma Annulare
Granuloma Faciale
Other Problems to Be Considered
Lupus erythematosus tumidus
Gyrate erythema
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.
Other Tests
- Phototesting: 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.
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.
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.4
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.
Treatment
Medical Care
Prognosis is good because lymphocytic infiltration of the skin 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. In 2008, 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.5
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.
Medication
The goals of pharmacotherapy are to reduce morbidity and prevent complications.
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.
Hydrocortisone valerate (Westcort)
Anti-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.
Dosing
Adult
Apply sparingly to affected areas bid
Pediatric
Not established
Interactions
None reported
Contraindications
Documented hypersensitivity; viral, fungal, and bacterial skin infections
Precautions
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Prolonged 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
Triamcinolone (Aristocort ointment, Kenalog cream)
For 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.
Dosing
Adult
2.5-10 mg/mL intralesionally; may be given IM in a concentration of 40 mg/mL; also comes as a 0.1% cream and ointment
Pediatric
<12 years: Not established
>12 years: Administer as in adults
Interactions
Coadministration with barbiturates, phenytoin, and rifampin decreases effects of triamcinolone
Contraindications
Documented hypersensitivity; fungal, viral, and bacterial skin infections
Precautions
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
If 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
Betamethasone dipropionate (Diprosone lotion)
For inflammatory dermatosis responsive to steroids; decreases inflammation. Available as 0.05% cream and ointment.
Dosing
Adult
Apply thin film bid until response
Pediatric
<12 years: Not recommended
Interactions
None reported
Contraindications
Documented hypersensitivity; viral, fungal, and bacterial skin infections
Precautions
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Can 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
Prednisone (Deltasone, Orasone)
For treating lymphocytic infiltration of the skin, 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.
Dosing
Adult
1-1.5 mg/kg/d initial qam or in divided doses; titrate dose to clinical response; taper PO steroids when disease is inactive
Pediatric
Administer as in adults
Interactions
Coadministration 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
Contraindications
Documented 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
Precautions
Pregnancy
B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
Precautions
Abrupt 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
Antimalarials
Used to treat certain photosensitive eruptions including lymphocytic infiltration of the skin.
Hydroxychloroquine (Plaquenil)
Mechanism of action not fully understood. Postulates include light filtration, immunosuppression, anti-inflammatory, and DNA binding. Supplied as 200-mg tabs.
Dosing
Adult
100-200 mg PO bid
Pediatric
10 mg base/kg/d PO qd or divided bid initially, followed by 5 mg/kg at 6, 24, and 48 h
Interactions
Cimetidine 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)
Contraindications
Documented 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
Precautions
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Caution 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
Follow-up
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 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.
Prognosis
- Prognosis is good because lymphocytic infiltration of the skin may resolve spontaneously.
Patient Education
- Provide education relating to the benign nature of lymphocytic infiltration of the skin.
Miscellaneous
Medicolegal Pitfalls
- Failing to exclude other conditions that may appear clinically similar to lymphocytic infiltration of the skin 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.
Multimedia
Media file 1: Jessner lymphocytic infiltration of the skin.
Media file 2: Jessner lymphocytic infiltration of the skin.
References
Jessner M, Kanof NB. Lymphocytic infiltration of the skin. Arch Dermatol. 1953;68:447-9.
Weber F, Schmuth M, Fritsch P, Sepp N. Lymphocytic infiltration of the skin is a photosensitive variant of lupus erythematosus: evidence by phototesting. Br J Dermatol. Feb 2001;144(2):292-6. [Medline].
Dippel E, Poenitz N, Klemke CD, Orfanos CE, Goerdt S. Familial lymphocytic infiltration of the skin: histochemical and molecular analysis in three brothers. Dermatology. 2002;204(1):12-6. [Medline].
Ashworth J, Turbitt M, MacKie R. A comparison of the dermal lymphoid infiltrates in discoid lupus erythematosus and Jessner's lymphocytic infiltrate of the skin using the monoclonal antibody Leu 8. J Cutan Pathol. Aug 1987;14(4):198-201. [Medline].
Borges da Costa J, Boixeda P, Moreno C. Pulsed-dye laser treatment of Jessner lymphocytic infiltration of the skin. J Eur Acad Dermatol Venereol. Sep 1 2008;[Medline].
Adamski H, Labrousse AL, Sparsa A, Leonard F, Le Gall F, Labrousse F, et al. [Positive photobiological investigation in Jessner's lymphocytic infiltration of the skin]. Ann Dermatol Venereol. Dec 2002;129(12):1370-3. [Medline].
Anderson NP, Newman BA, Feldman FF. Lymphocytic infiltration of the skin. Arch Dermatol. 1954;70:832.
Arai E, Shimizu M, Hirose T. A review of 55 cases of cutaneous lymphoid hyperplasia: reassessment of the histopathologic findings leading to reclassification of 4 lesions as cutaneous marginal zone lymphoma and 19 as pseudolymphomatous folliculitis. Hum Pathol. May 2005;36(5):505-11. [Medline].
Braun-Falco O, Plewig G, Wolff HH. Pseudolymphomas of the skin. Dermatology. 1984;1074-5.
Calnan CD. Lymphocytic infiltration of the skin (Jessner). Br J Dermatol. May 1957;69(5):169-73. [Medline].
Gottlieb M, Winkelman RK. Lymphocytic infiltration of the skin. Arch Dermatol. 1962;68:626.
Hafejee A, Winhoven S, Coulson IH. Jessner's lymphocytic infiltrate responding to oral auranofin. J Dermatolog Treat. Sep 2004;15(5):331-2. [Medline].
Kuhn A, Richter-Hintz D, Oslislo C, Ruzicka T, Megahed M, Lehmann P. Lupus erythematosus tumidus--a neglected subset of cutaneous Lupus erythematosus: report of 40 cases. Arch Dermatol. Aug 2000;136(8):1033-41. [Medline].
Lever WF, Lever GS. Connective Tissue Diseases. In: Histopathology of the Skin. Baltimore, Md: Lippincott Williams & Wilkins; 1990:505-06.
Mackie RM. Cutaneous Lymphomas and Lymphocytic Infiltrates. In: Textbook of Dermatology. Vol. 3. London, England: Blackwell Science; 1998:2400-01.
Nolden S, Casper C, Kuhn A, Petereit HF. Jessner-Kanof lymphocytic infiltration of the skin associated with glatiramer acetate. Mult Scler. Apr 2005;11(2):245-8. [Medline].
Poenitz N, Dippel E, Klemke CD, Qadoumi M, Goerdt S. Jessner's lymphocytic infiltration of the skin: a CD8+ polyclonal reactive skin condition. Dermatology. 2003;207(3):276-84. [Medline].
Rijlaarsdam JU, Nieboer C, de Vries E, Willemze R. Characterization of the dermal infiltrates in Jessner's lymphocytic infiltrate of the skin, polymorphous light eruption and cutaneous lupus erythematosus: differential diagnostic and pathogenetic aspects. J Cutan Pathol. Feb 1990;17(1):2-8. [Medline].
Toonstra J, Wildschut A, Boer J, Smeenk G, Willemze R, van der Putte SC, et al. Jessner's lymphocytic infiltration of the skin. A clinical study of 100 patients. Arch Dermatol. Nov 1989;125(11):1525-30. [Medline].
Willemze R, Dijkstra A, Meijer CJ. Lymphocytic infiltration of the skin (Jessner): a T-cell lymphoproliferative disease. Br J Dermatol. May 1984;110(5):523-9. [Medline].
Wolf M. Lymphocytic infiltration of the face. Arch Dermatol. 1957;75:136.
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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
Contributor Information and Disclosures
Author
Bassam Zeina, MD, PhD, Consulting Staff, Department of Dermatology, Milton Keynes Hospital, UK
Bassam Zeina, MD, PhD is a member of the following medical societies: British Association of Dermatologists, British Medical Association, and Royal Society of Medicine
Disclosure: Nothing to disclose.
Coauthor(s)
Sohail Mansoor, MBBS, MSc, Dermatologist and Lead Physician in Dermatologic Surgery, Department of Dermatology, Barnet Hospital, UK
Sohail Mansoor, MBBS, MSc is a member of the following medical societies: American Academy of Anti-Aging Medicine, American Academy of Dermatology, American Society for Dermatologic Surgery, Royal College of Physicians and Surgeons of Glasgow, and Royal College of Physicians of the United Kingdom
Disclosure: Nothing to disclose.
Medical Editor
Peter Fritsch, MD, Chair, Department of Dermatology and Venereology, University of Innsbruck, Austria
Peter Fritsch, MD is a member of the following medical societies: American Dermatological Association, International Society of Pediatric Dermatology, and Society for Investigative Dermatology
Disclosure: Nothing to disclose.
Pharmacy Editor
Richard P Vinson, MD, Assistant Clinical Professor, Department of Dermatology, Texas Tech University School of Medicine; Consulting Staff, Mountain View Dermatology, PA
Richard P Vinson, MD is a member of the following medical societies: American Academy of Dermatology, Association of Military Dermatologists, Texas Dermatological Society, and Texas Medical Association
Disclosure: Nothing to disclose.
Managing Editor
Daniel S Loo, MD, Associate Professor of Dermatology, Residency Program Director, Department of Dermatology, Tufts Medical Center
Daniel S Loo, MD is a member of the following medical societies: American Academy of Dermatology and Association of Professors of Dermatology
Disclosure: Nothing to disclose.
CME Editor
Glen H Crawford, MD, Assistant Clinical Professor, Department of Dermatology, University of Pennsylvania School of Medicine; Chief, Division of Dermatology, The Pennsylvania Hospital
Glen H Crawford, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, Phi Beta Kappa, and Society of USAF Flight Surgeons
Disclosure: Nothing to disclose.
Chief Editor
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
William D James, MD is a member of the following medical societies: American Academy of Dermatology and Society for Investigative Dermatology
Disclosure: elsevier Royalty Other; american college of physicians Honoraria Other