Lymphocytoma Cutis

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Author

Christine J Ko, MD, Assistant Professor, Departments of Dermatology and Pathology, Yale University School of Medicine

Nothing to disclose.

Coauthor(s)

Earl J Glusac, MD, Professor, Departments of Pathology and Dermatology, Yale University School of Medicine

Nothing to disclose.

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

Nothing to disclose.

Specialty Editor(s)

Catherine M Quirk, MD, Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania

Nothing to disclose.

Daniel S Loo, MD, Associate Professor of Dermatology, Residency Program Director, Department of Dermatology, Tufts Medical Center

Nothing to disclose.

Günter Burg, MD, Professor and Chairman Emeritus, Department of Dermatology, University of Zürich School of Medicine; Delegate of The Foundation for Modern Teaching and Learning in Medicine Faculty of Medicine, University of Zürich, Switzerland

Nothing to disclose.

Richard P Vinson, MD, Assistant Clinical Professor, Department of Dermatology, Texas Tech University School of Medicine; Consulting Staff, Mountain View Dermatology, PA

Nothing to disclose.

Chief Editor

Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center

Nothing to disclose.

Background

Lymphocytoma cutis is not a specific disease. Rather, lymphocytoma cutis 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, lymphocytoma cutis 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. Note the images below.


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This example of lymphocytoma cutis shows a localized, erythematous-to-brown, ill-defined plaque.


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Lymphocytoma cutis of the shoulder, composed of flesh-colored or erythematous nodules in small groups.


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This photograph of lymphocytoma cutis caused by an arthropod bite shows an erythematous scaling patch of the scalp with localized secondary alopecia.

Related eMedicine articles include Cutaneous B-Cell Lymphoma and Cutaneous T-Cell Lymphoma.

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 of lymphocytoma cutis 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.[1]

Epidemiology

Frequency

United States

No data on US prevalence or incidence of lymphocytoma cutis are available.

International

No data are available. Lymphocytoma cutis 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. Lymphocytoma cutis is rarely associated with morbidity other than minor pain or pruritus. It generally heals without scarring.

Race

No racial predilection is noted for lymphocytoma cutis.

Sex

The female-to-male ratio in reported of lymphocytoma cutis cases is approximately 2:1.

Age

Individuals of any age may be affected, but lymphocytoma cutis is most common in early adulthood.

History

Physical

Causes

Most cases of lymphocytoma cutis are idiopathic. Known inciting agents for lymphocytoma cutis include the following:

Laboratory Studies

Other Tests

Histologic Findings

Lymphocytoma cutis must be differentiated from lymphoma. Most cases of lymphocytoma cutis have a nodular inflammatory infiltrate and can simulate B-cell lymphoma.[9] 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.[10] In lymphocytoma cutis, the infiltrate tends to be top-heavy, whereas most lymphomas are centered in the deep dermis or subcutis. Samples of lymphocytoma cutis typically have germinal centers and tingible body macrophages, as shown in the images below. Occasionally, large lymphoid cells may be present; however, they rarely dominate the histologic picture.


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A mixed inflammatory infiltrate with germinal centers is indicative of lymphocytoma cutis.


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Well-developed lymphoid follicles in a background of mixed inflammatory cells with small lymphocytes are typical of lymphocytoma cutis.

Immunohistochemical staining may also be useful and generally shows a mixed B- and T-cell population with a high MIB-1–positive proliferative index. 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 of lymphocytoma cutis 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.[11]

Medical Care

Surgical Care

Surgical removal or cryosurgery may be effective in some cases of lymphocytoma cutis.

Complications

Prognosis

References

  1. Colli C, Leinweber B, Mullegger R, Chott A, Kerl H, Cerroni L. Borrelia burgdorferi-associated lymphocytoma cutis: clinicopathologic, immunophenotypic, and molecular study of 106 cases. J Cutan Pathol. Mar 2004;31(3):232-40.[View Abstract]
  2. Kazandjieva J, Tsankov N. Tattoos: dermatological complications. Clin Dermatol. Jul-Aug 2007;25(4):375-82.[View Abstract]
  3. Albrecht J, Fine LA, Piette W. Drug-associated lymphoma and pseudolymphoma: recognition and management. Dermatol Clin. Apr 2007;25(2):233-44, vii.[View Abstract]
  4. Welsh JP, Ko C, Hsu WT. Lymphomatoid drug reaction secondary to methylphenidate hydrochloride. Cutis. Jan 2008;81(1):61-4.[View Abstract]
  5. Porto DA, Comfere NI, Myers LM, Abbott JJ. Pseudolymphomatous reaction to varicella zoster virus vaccination: role of viral in situ hybridization. J Cutan Pathol. Nov 4 2009;[View Abstract]
  6. Kempf W, Dummer R, Burg G. Approach to lymphoproliferative infiltrates of the skin. The difficult lesions. Am J Clin Pathol. Jan 1999;111(1 Suppl 1):S84-93.[View Abstract]
  7. Wantzin GL, Hou-Jensen K, Nielsen M, Petri J, Thomsen K. Cutaneous lymphocytomas: clinical and histological aspects. Acta Derm Venereol. 1982;62(2):119-24.[View Abstract]
  8. van Vloten WA, Willemze R. The many faces of lymphocytoma cutis. J Eur Acad Dermatol Venereol. Jan 2003;17(1):3-6.[View Abstract]
  9. Duncan SC, Evans HL, Winkelmann RK. Large cell lymphocytoma. Arch Dermatol. Oct 1980;116(10):1142-6.[View Abstract]
  10. Geerts ML, Kaiserling E. A morphologic study of lymphadenosis benigna cutis. Dermatologica. 1985;170(3):121-7.[View Abstract]
  11. Ploysangam T, Breneman DL, Mutasim DF. Cutaneous pseudolymphomas. J Am Acad Dermatol. Jun 1998;38(6 Pt 1):877-95; quiz 896-7.[View Abstract]

This example of lymphocytoma cutis shows a localized, erythematous-to-brown, ill-defined plaque.

Lymphocytoma cutis of the shoulder, composed of flesh-colored or erythematous nodules in small groups.

This photograph of lymphocytoma cutis caused by an arthropod bite shows an erythematous scaling patch of the scalp with localized secondary alopecia.

A mixed inflammatory infiltrate with germinal centers is indicative of lymphocytoma cutis.

Well-developed lymphoid follicles in a background of mixed inflammatory cells with small lymphocytes are typical of lymphocytoma cutis.