You are in: eMedicine Specialties > Dermatology > LYMPHOMA AND RELATED PROCESSES Jessner Lymphocytic Infiltration of the SkinArticle Last Updated: Oct 17, 2007AUTHOR AND EDITOR INFORMATIONAuthor: 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 INTRODUCTIONBackgroundJessner 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 PathophysiologyWhether 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:
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. FrequencyUnited StatesThe frequency of this condition in the United States is unknown. InternationalThe incidence and prevalence internationally is unknown. It is considered uncommon. Mortality/MorbidityLIS is not associated with increased mortality. The lesions are commonly asymptomatic, although some patients report burning or pruritus. RaceLIS has no known racial predilection. SexThe 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. AgeLIS affects mostly adults younger than 50 years. It has been reported in children. Familial occurrence has been reported. CLINICALHistoryPatients 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. PhysicalPertinent physical findings are limited to the skin.
CausesThe 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. DIFFERENTIALSGranuloma Annulare Granuloma Faciale
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| Drug Name | Hydrocortisone valerate (Westcort) |
|---|---|
| Description | 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. |
| Adult Dose | Apply sparingly to affected areas bid |
| Pediatric Dose | Not established |
| Contraindications | Documented hypersensitivity; viral, fungal, and bacterial skin infections |
| Interactions | None reported |
| 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 |
| Drug Name | Triamcinolone (Aristocort ointment, Kenalog cream) |
|---|---|
| Description | 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. |
| Adult Dose | 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 Dose | <12 years: Not established >12 years: Administer as in adults |
| Contraindications | Documented hypersensitivity; fungal, viral, and bacterial skin infections |
| Interactions | Coadministration with barbiturates, phenytoin, and rifampin decreases effects of triamcinolone |
| 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 |
| Drug Name | Betamethasone dipropionate (Diprosone lotion) |
|---|---|
| Description | For inflammatory dermatosis responsive to steroids; decreases inflammation. Available as 0.05% cream and ointment. |
| Adult Dose | Apply thin film bid until response |
| Pediatric Dose | <12 years: Not recommended |
| Contraindications | Documented hypersensitivity; viral, fungal, and bacterial skin infections |
| Interactions | None reported |
| 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 |
| Drug Name | Prednisone (Deltasone, Orasone) |
|---|---|
| Description | For 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 Dose | 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 Dose | Administer as in adults |
| 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 |
| 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 |
| 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 |
Used to treat certain photosensitive eruptions including LIS.
| Drug Name | Hydroxychloroquine (Plaquenil) |
|---|---|
| Description | Mechanism of action not fully understood. Postulates include light filtration, immunosuppression, anti-inflammatory, and DNA binding. Supplied as 200-mg tabs. |
| Adult Dose | 100-200 mg PO bid |
| Pediatric Dose | 10 mg base/kg/d PO qd or divided bid initially, followed by 5 mg/kg at 6, 24, and 48 h |
| 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 |
| 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) |
| 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 |
| 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