|55 Kenosia Avenue
Danbury, CT 06810
Toll Free: 1.800.999.6673
The National Organization for Rare Disorders (NORD) web site, its databases, and the contents thereof are copyrighted by NORD. No part of the NORD web site, databases, or the contents may be copied in any way, including but not limited to the following: electronically downloading, storing in a retrieval system, or redistributing for any commercial purposes without the express written permission of NORD. Permission is hereby granted to print one hard copy of the information on an individual disease for your personal use, provided that such content is in no way modified, and the credit for the source (NORD) and NORD’s copyright notice are included on the printed copy. Any other electronic reproduction or other printed versions is strictly prohibited.
The information in NORD’s Rare Disease Database is for educational purposes only. It should never be used for diagnostic or treatment purposes. If you have questions regarding a medical condition, always seek the advice of your physician or other qualified health professional. NORD’s reports provide a brief overview of rare diseases. For more specific information, we encourage you to contact your personal physician or the agencies listed as “Resources” on this report.
Copyright 1987, 1989, 1990, 1992, 1997, 2004
Jessner lymphocytic infiltrate of the skin is an uncommon disorder that is characterized by benign accumulations of lymph cells in the skin. These small lesions are solid, pink or red in color, and appear on the face, neck, and/or back. Skin surrounding these lesions may be itchy and turn red. The lesions may remain unchanged for several years and then spontaneously disappear, leaving no scars.
Not much is known about this disorder. In fact, there is some difference of opinion as to whether it is distinguishable as a separate condition or represents a stage of some other disorder. Some scientists believe that Jessner lymphocytic infiltrate may be a type of lupus erythematosus tumidus (LET) or discoid lupus erythematosus (DLE).
Symptoms of Jessner lymphocytic infiltrate of the skin include rosy-red, pimple-like eruptions, most commonly on the upper face, cheeks and neck. These lesions are non-scaly in nature and may last for several months. They often expand from the perimeter to form well-defined, smooth, red plaques that vary in diameter up to about one inch.
Occasionally, clear centers will form on the plaques. The skin surrounding the lesions may be reddened and itching. Sensitivity to sunlight may occur but is unusual. Generally, the symptoms disappear after several years but it is possible that they may reappear later. Sometimes, they go through times of getting worse and then getting better again over a period of months or years. Sometimes, they simply go away for no apparent reason.
The exact cause of Jessner lymphocytic infiltrate is not known. Abnormal numbers of lymph cells accumulate in the skin for no clear reason. It is not even clear that this is a disorder distinct from other benign infiltrates of the skin.
Clinical researchers have proposed that Jessner lymphocytic infiltrate may be any one of the following: a distinct entity or disorder in all cases, a distinct entity in some cases but lupus erythematosus (DLE) in others, either DLE or LET (a sub-type of DLE) in all cases, or an initial stage (or an intermediate stage) in the development of some other disorder.
The incidence or prevalence of Jessner lymphocytic infiltrate of the skin in the United States or Europe is not known. The disorder appears to affect adults before their fifth decade, i.e. younger than 50 years of age. Rarely, it affects children. Although there have been some cases in which it appeared that more than one sibling or family member was affected, no genetic component has been isolated.
Symptoms of the following disorders can be similar to Jessner lymphocytic infiltrate of the skin. Comparisons may be useful for a differential diagnosis:
Lymphocytoma cutis is characterized by a skin nodule caused by dense accumulation of lymph cells and histiocytes which are cells that are usually found in connective tissue. These lesions often form purplish, yellow-brown, glistening, spherical masses. They may be widespread or limited to a small area, and are separated from the outer layer of the skin (epidermis) by a narrow noninfiltrating layer.
Mycosis fungoides is a chronic progressive lymph disorder which initially resembles eczema or other inflammatory skin disorders. Later, tumors resembling mushrooms tend to appear on the skin. Thickened layers of skin (acanthosis) and a band-shaped infiltration of the upper skin layer by two kinds of atypical lymphoid cells occurs. In advanced cases, ulcerated tumors and cellular infiltrations of lymph nodes may occur. (For more information on this disorder, choose "Mycosis Fungoides" as your search term in the Rare Disease Database.)
Discoid lupus erythematosus is a form of lupus erythematosus in which only skin lesions are present. These degenerative plaques commonly appear on the face causing redness, roughness (hyperkeratosis), plugged hair follicles, and dilated blood vessels (telangiectasia). In some cases, this disorder can progress to systemic lupus erythematosus. (For more information on these disorders, choose "Lupus" as your search term in the Rare Disease Database.)
Leprosy (Hansen's disease) is a progressive, contagious disorder usually found in warmer climates and characterized by abnormal cell (granulomatous) formations around the nerves in the skin. It is caused by a type of airborne bacteria known as Hansen's bacillus (Mycobacterium leprae). (For more information on this disorder, choose "Leprosy" as your search term in the Rare Disease Database.)
A skin biopsy, using skin from a recently formed lesion, begins the process of excluding other possible conditions. Further relatively sophisticated tests on the biopsy materials are then undertaken to focus on a final diagnosis.
A period of watchful waiting is recommended to see if the disorder will clear up spontaneously. Treatment includes the use of cosmetics, protection from sunlight, cutting out small lesions surgically, topical medium-potency steroids and other steroids, freezing lesions (cryotherapy), and oral hydroxychloroquine. The use of steroids, topical or systemic, must be monitored carefully, as must the use of hydroxychloroquine.
Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. government funding, and some supported by private industry, are posted on this government web site.
For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:
Tollfree: (800) 411-1222
TTY: (866) 411-1010
For information about clinical trials sponsored by private sources, go to:
PO Box 8126
Gaithersburg, MD 20898-8126
Phone #: 301-251-4925
800 #: 888-205-2311
Home page: http://rarediseases.info.nih.gov/GARD/
One AMS Circle
Bethesda, MD 20892-3675 USA
Phone #: 301-495-4484
800 #: 877-226-4267
Home page: http://www.niams.nih.gov/
Champion RH, Burton JL, Ebling FJG, eds. Textbook of Dermatology. 5th ed. Blackwell Scientific Publications. London, UK; 1992:2103-04.
Poenitz N, Dippel E, Klemke CD, et al. Jessner lymphocytic infiltrate of the skin: a CD8+ polyclonal reactive skin condition. Dermatology. 2003;207:276-84.
Colburn DE, Welch MA, Giles FJ. Skin infiltration with chronic lymphocytic leukemia is consistent with a good prognosis. Hematology. 2002;7:187-88.
Dippel E, Poenitz N, Klemke CD, et al. Familial lymphocytic infiltration of the skin: histochemical and molecular analysis in three brothers. Dermatology. 2002;204:12-16.
Weber F, Schmuth M, Fritsch P, et al. Lymphocytic infiltration of the skin is a photosensitive variant of lupus erythematosus: evidence by phototesting. Br J Dermatol. 2001;144:292-96.
Kunz M, Henseleit-Walter U, Sorg K, et al. Macrophage marker 27E10 on human keratinocytes helps to differentiate discoid lupus erythematosus and Jessner's lymphocytic infiltrate of the skin. Eur J Dermatol. 1999;9:107-10.
Guillaume JC, Moulin G, Dieng MT et al. Crossover study of thalidomide vs placebo in Jessner's lymphocytic infiltrate of the skin. Arch Dermatol. 1995;131:1032-35.
Toonstra I, van der Putte SC, de la faille HB, et al. Familial Jessner's lymphocytic infiltrate of the skin, occurring in a father and daughter. Clin Exp Dermatol. 1993;18:142-45.
FROM THE INTERNET
Zeina B. Jessner Lymphocytic Infiltration of the Skin. emedicine. Last Updated: February 11, 2004. 11pp.
Report last updated: 2008/05/21 00:00:00 GMT+0