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Lymphocytoma Cutis
Article Last Updated: Sep 12, 2006
AUTHOR AND EDITOR INFORMATION
Section 1 of 9  
Author: Earl Glusac, MD, Professor, Departments of Pathology and Dermatology, Yale University School of Medicine
Earl Glusac is a member of the following medical societies: American Academy of Dermatology
Coauthor(s):
Jon H Meyerle, MD, Assistant Professor, Department of Dermatology, Johns Hopkins University School of Medicine; Consulting Staff, Laboratory Director, Department of Dermatology, Walter Reed Army Medical Center and National Naval Medical Center
Editors: Günter Burg, MD, Professor and Chairman Emeritus, Department of Dermatology, University of Zürich School of Medicine, Switzerland; 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; Catherine Quirk, MD, Clinical Assistant Professor, Department of Dermatology, Brown University; Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center
Author and Editor Disclosure
Synonyms and related keywords:
cutaneous B- or T-cell pseudolymphoma, cutaneous lymphoid hyperplasia, pseudolymphoma without modification, pseudolymphoma of Spiegler and Fendt, sarcoidosis of Spiegler and Fendt, cutaneous lymphomatous hyperplasia, lymphadenosis benigna cutis, cutaneous lymphoplasia, Ixodes ricinus, Borrelia burgdorferi subsp afzelius, varicella zoster, molluscum contagiosum
Background
Lymphocytoma cutis is not a specific disease. Rather, it is a response to a variety of known and unknown stimuli that results in the accumulation of lymphocytes and other inflammatory cells in a localized region. Lymphocytoma cutis is known to simulate lymphoma, as reflected primarily by the histologic findings. However, it is a reactive process and generally has a benign course. When known, the inciting agent should be included in the diagnosis. The term lymphocytoma cutis or pseudolymphoma without modification should be reserved for idiopathic cases.
Pathophysiology
In lymphocytoma cutis, lymphocytes and other inflammatory cells are recruited to a localized area of the skin in response to known or unknown stimuli. Most cases are idiopathic. Lymphocytoma cutis is not considered a lymphocytic response to malignancy.
Cases with known etiologies involve a reaction to tattoo dye, jewelry (especially gold), arthropod bites, medications, folliculitis, trauma, infections (ie, with Borrelia burgdorferi, molluscum contagiosum), vaccinations, and acupuncture.
A discrete subset of lymphocytoma cutis, borrelial lymphocytoma, occurs primarily in Europe, in areas in which the Ixodes ricinus tick is endemic. This form of the disease is a response to an infection due to Borrelia burgdorferi subsp afzelius that is transmitted by a tick bite.
Frequency
United States
No data are available.
International
No data are available. The condition is uncommon but not rare. A discrete subset of lymphocytoma cutis, borrelial lymphocytoma, occurs primarily in Europe, in areas in which the I ricinus tick is endemic.
Mortality/Morbidity
Lymphocytoma cutis is not associated with mortality. It is rarely associated with morbidity other than minor pain or pruritus. It generally heals without scarring.
Race
No racial predilection is noted.
Sex
The female-to-male ratio in reported cases is approximately 2:1.
Age
Individuals of any age may be affected, but lymphocytoma cutis is most common in early adulthood.
- The mean patient age at onset is 34 years. Approximately 8% of cases involve patients younger than 18 years.
- Two thirds of patients are younger than 40 years at the time of biopsy.
- Borrelial lymphocytoma is more common in children than adults and is most often observed in Europe.
History
- Patients present with a nodule or group of discrete nodules, usually with minimal associated symptoms.
- Occasionally, pruritus or pain is noted.
Physical
- Examination usually reveals a single nodule with a diameter of one to several centimeters.
- Although the lesions may be soft, they are more often firm.
- Typically, the lesions are red to purple but can be absent of color as well.
- Approximately three quarters of the cases involve localized lesions.
- The remaining cases usually involve grouped papules in a single defined region.
- More disseminated cases are rare.
- The most common site of involvement is the face (70%).
- Next most common sites are the chest and upper extremities.
- Lesions below the waist are uncommon.
- Sites of predilection for Borrelial lymphocytoma are sites with low skin temperature, including the following:
- Earlobes
- Nipples and areolae
- Nose
- Scrotum
Causes
Most cases are idiopathic. Known inciting agents include the following:
- Tattoo dye
- Jewelry (eg, gold earrings)
- Arthropod (insect and spider) bites
- Medications
- Folliculitis
- Trauma
- Vaccinations
- Materials that come into direct contact with the skin or are injected into the skin (eg, gold, aluminum)
- Infection (eg, varicella-zoster virus, Borrelia species, molluscum contagiosum, Helicobacter pylori)
- Sites where acupuncture needles were inserted
Lymphomatoid Papulosis
Syphilis
Other Problems to be Considered
Lymphoma cutis
Lymphocytic infiltrate of Jessner
Basal cell epithelioma or other adnexal tumors
Malignant B-cell lymphoma (Transformation into malignant B-cell lymphoma is reported.)
Lab Studies
- Biopsy is necessary to establish a diagnosis of lymphocytoma and exclude cutaneous lymphoma.
- Adequate sample is essential. The specimen should be obtained by extending well into the subcutis while avoiding crush artifact.
Other Tests
- In some cases, the possibility of lymphoma cannot be excluded by means of histologic analysis. In such cases, analysis for immunoglobulin or T-cell receptor genetic rearrangements may provide additional helpful information.
- If a clone is identified, it increases the likelihood of lymphoma.
- The results of this test should not be considered definitive.
- Clonality is reported in occasional pseudolymphomas and may be absent in some lymphoma samples.
- Clinical-pathologic correlation remains the criterion standard in the differential diagnosis.
- Antibodies to B burgdorferi may be identified in 50% of patients with borrelial lymphocytoma. The organism can be identified in tissue by means of polymerase chain reaction analysis.
Histologic Findings
Lymphocytoma cutis must be differentiated from lymphoma. Most cases of lymphocytoma cutis simulate B-cell lymphoma. The key histologic features that suggest lymphocytoma cutis instead of lymphoma include the presence of a mixed infiltrate that includes histiocytes, eosinophils, and plasma cells, in addition to lymphocytes. In lymphocytoma cutis, the infiltrate tends to be superficial, whereas most lymphomas are centered in the deep dermis or subcutis. Samples of lymphocytoma cutis typically have germinal centers and tingible body macrophages (see Image 5). Occasionally, large lymphoid cells may be present; however, they rarely dominate the histologic picture. Immunohistochemical staining may also be useful and generally shows a mixed B-cell and T-cell population. Staining for kappa and lambda light chains reveals a polyclonal pattern. Fresh, unfixed tissue may be required for adequate assessment of kappa and/or lambda labeling. Some cases have a T-cell histologic pattern. Changes include a bandlike infiltrate in the papillary dermis, which predominantly includes small lymphocytes with variable epidermotropism. Although these features mimic those of cutaneous T-cell lymphoma or mycosis fungoides, the clinical presentation is characteristic.
Medical Care
- When the offending agent is known, its removal results in disease resolution.
- Cases that occur as a result of infection should be treated appropriately.
- In idiopathic cases, treatment is not mandatory.
- Cures may be achieved by local radiation therapy.
- Some report a response to topical or injected corticosteroids as well as topical immunomodulators such as tacrolimus.
- Patients with presumed lymphocytoma cutis in whom lymphoma cannot be excluded should be examined for the possibility of concurrent extracutaneous disease. Careful follow-up is required to evaluate the possible emergence of lymphoma.
Surgical Care
Surgical removal or cryosurgery may be effective in some cases.
Complications
- Rare case reports describe the evolution to lymphoma.
- Histologically, a significant percentage of these cases may have represented lymphoma from the outset.
Prognosis
- Spontaneous regression often occurs.
- If the inciting agent is removed, the condition resolves.
- Idiopathic cases tend to be chronic and indolent.
- Local recurrence has been noted.
Patient Education
| Media file 1:
This example of lymphocytoma cutis shows a localized, erythematous-to-brown, ill-defined plaque. |
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| Media file 2:
Lymphocytoma cutis of the shoulder, composed of flesh-colored or erythematous nodules in small groups. |
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| Media file 3:
This photograph of lymphocytoma cutis caused by an arthropod bite shows an erythematous scaling patch of the scalp with localized secondary alopecia. |
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| Media file 4:
A mixed inflammatory infiltrate with germinal centers is indicative of lymphocytoma cutis. |
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| Media file 5:
Well-developed lymphoid follicles in a background of mixed inflammatory cells with small lymphocytes are typical of lymphocytoma cutis. |
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Lymphocytoma Cutis excerpt Article Last Updated: Sep 12, 2006
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