Jessner’s Lymphocytic Infiltration of the Skin - An Overview

Introduction

Jessner’s lymphocytic infiltrate of the skin (JLIS) is a rare skin condition. It is characterized by papular or plaque-like eruptions. A papule is a raised skin area. When the papule coalesces, a plaque is formed. It usually involves sun-exposed areas such as the neck, face, and the chest. JLIS has a slow course. Hence, the lesions last from weeks to months. The disease usually does not have any symptoms.

What Are the Causes of Jessners Lymphocytic Infiltrate of the Skin?

The exact cause of JLIS remains unknown. However, JLIS has some proposed reasons.

1. Genetic: Studies have reported familial cases of JLIS. Hence, a hereditary factor is one of the causes.

2. Autoimmune: The dendritic cells of the skin play a crucial role in the etiology. Dendritic cells are immune cells that are involved in regulating the immune response. It has been postulated that these cells play a role in this disease, similar to systemic lupus erythematosus (SLE, an autoimmune disease). A hypothesis is that JLIS is an early manifestation of SLE.

3. Photosensitivity: Another trigger is sun exposure. The lesions show a similar pattern to the polymorphous light eruption (PMLE, a rash caused due to sun exposure). Hence, this disease is linked to PMLE.

4. Drug-Induced: Medicines are also implicated in causing the condition. Angiotensin-converting enzyme inhibitors (ACE-I, a class of antihypertensives), antidepressants (Duloxetine), and immunosuppressants (Etanercept, Ustekinumab, Leflunomide, and Glatiramer acetate) can cause JLIS.

5. Infections: Some studies have reported human immunodeficiency virus (HIV) and Borrelia burgdorferi (a bacterium) causing JLIS.

How Common Is Jessners Lymphocytic Infiltrate of the Skin?

The incidence (new cases in a given time) and prevalence (how common is the disease) is unknown. The condition affects individuals younger than age 50. Some studies report a male predisposition. However, there is no reported ethnic predilection.

What Are the Signs and Symptoms of Jessners Lymphocytic Infiltrate of the Skin?

Patients with JLIS have reddish papules and plaques, mainly on the face, neck, upper back, and chest. Extremities may become involved later. Some patients experience pruritis (itching). There is a history of spontaneous disease occurrence and resolution with symptom disappearance. The lesions are well-defined and expand peripherally (away from the body’s center). Lesions range from two millimeters to two centimeters in size. Single or multiple lesions are found. One complication of JLIS is that the lesions can progress into skin atrophy (degeneration).

What Is the Histopathology of Jessners Lymphocytic Infiltrate of the Skin?

Histopathology (microscopy) shows a dense lymphocytic infiltration of the skin. Lymphocytes are white blood cells involved in the body’s defense. They are typically found surrounding the blood vessels. Lymphocytes can also penetrate deeper into the subcutaneous tissue. Both types of lymphocytes (B and T) are involved. Plasmacytoid dendritic cells are a type of immune cell that also get activated. They are scattered throughout the skin. Natural killer cells (another type of immune cells) are also triggered.

How Is the Diagnosis of Jessners Lymphocytic Infiltrate of the Skin Made?

A medical history and detailed physical examination are necessary. History can indicate the genetic involvement and association with sun exposure. A clinical workup is also warranted. Various investigations are:

1. Routine Investigations: Blood (complete blood count), urine, and fecal examination are done. Erythrocyte sedimentation rate (ESR) is also performed.

2. Autoimmune Workup: Antinuclear antibodies (ANA) are detected through enzyme assays such as enzyme-linked immunosorbent assay (ELISA). If the test is positive, further investigations for SLE are done (detection of anti-Smith and double-stranded-DNA antibodies). Other diseases should also be ruled out, such as Sjogren’s syndrome (anti-Ro and anti-La antibodies), antiphospholipid syndrome (lupus anticoagulant), and rheumatoid arthritis (rheumatoid factor).

3. Biopsy: Skin biopsy is diagnostic. It is done with hematoxylin and eosin (H and E) staining. Direct immunofluorescence is also done (negative in JLIS).

4. Photo Testing: Provocative photo testing is performed for diagnostic purposes. It is done with ultraviolet radiation (UVR) in patients with sun-exposure lesions. UV-A and UB-B are used in the test. The forearm is exposed to UV-B light with a fluorescent lamp for three consecutive days. A similar area on the other arm is exposed to UV-A light. The photosensitive sites on the arms are exposed. A skin biopsy is done if a rash develops.

What Is the Treatment of Jessners Lymphocytic Infiltrate of the Skin?

There is no definitive treatment for JLIS. It is because the lesions resolve spontaneously. Hence, careful monitoring is advised. However, medications and specific treatment modalities have been advocated for the management.

1. Medications:

• Corticosteroids: These work by modifying the body’s immune response and reducing inflammation. Corticosteroids are the first-line treatment for JLIS. They are given in topical (direct application), intralesional injection (into the lesion), and oral forms. Hydrocortisone valerate is available as a 0.2 percent cream. Betamethasone is also available as a topical medication (0.05 percent ointment). Another option is triamcinolone acetonide. It can also be given intralesionally. Oral Prednisone is given for widespread lesions.

• Antimalarials: Hydroxychloroquine works through poorly understood mechanisms. The proposed theories are immunosuppression, DNA binding, and anti-inflammatory action. It is available as oral tablets (200 milligrams). Antimalarials are particularly effective in photosensitive patients.

2. Specific Treatments: Patients can opt for cosmetic treatments to remove the lesions.

• Camouflage: A patient applies creams, lotions, or powders to hide the skin lesions. It is also called medical makeup.

• Surgery: Small lesion excision is done via surgery.

• Cryotherapy: It is a minimally invasive treatment. It works by freezing the lesions with extremely cold liquids or instruments. The lesion usually falls after one week to ten days after therapy.

• Laser: Michel et al. (2010) described the use of a pulsed dye laser (PDL) for JLIS in five patients nonresponsive to steroids. The authors reported an excellent outcome in all five patients. They concluded that PDL could be a promising treatment option for JLIS. They further added that it could become a first-line treatment as it has no side effects.

• Photoprotection and Photodynamic Therapy: Photoprotection and photodynamic therapy have also been tried for the same.

3. Others: Chemotherapy drugs (Methotrexate and Cyclophosphamide) are advised. Thalidomide and Tacrolimus are immunosuppressants used for the same. Other drugs are Etretinate (a retinoid), oral Auranofin (an anti-inflammatory drug), and Proquazone. Proquazone is a non-steroidal anti-inflammatory drug (NSAID). It has anti-inflammatory, analgesic, and antipyretic properties.

Close observation after prescribing steroids is mandatory. A follow-up is also advocated to monitor the treatment progress. The prognosis of JLIS is good as it is a benign condition. Also, it has not been associated with an increased death rate.

Conclusion

The holistic care of the condition involves a close collaboration between the patient and the healthcare professionals. An important point for patients is to avoid sun exposure. It is important to explain all the available treatments and their side effects to the patients. However, there are no adequate preventive measures for the same.