Pseudolymphoma, Cutaneous

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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

Pseudolymphoma is not a specific disease but rather an inflammatory response to known or unknown stimuli that results in a lymphomatous-appearing but benign accumulation of inflammatory cells.[1] In cutaneous pseudolymphoma, resemblance to lymphoma is usually most apparent histologically, but some examples may also mimic lymphoma clinically. When known, the inciting agent should be included within the diagnosis of cutaneous pseudolymphoma. The term pseudolymphoma without modification should be reserved for idiopathic cases.

Localized, nodular pseudolymphomas are more common and typically mimic B-cell lymphoma (for further discussion, see Lymphocytoma Cutis). A variety of specific diseases are sometimes referred to as pseudolymphomas simply because they may resemble lymphoma. These disorders often show broad patches and plaques and often mimic cutaneous T-cell lymphoma. Examples include actinic reticuloid, lymphomatoid contact dermatitis, and lymphomatoid drug eruptions.[2] Note the images below.


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Pseudolymphomatous drug eruption due to captopril, marked by erythematous to purple papules, patches, and plaques.


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This erythrodermic pseudolymphoma (T-cell pattern) typifies drug-induced pseudolymphoma, which is most often secondary to anticonvulsant therapy.

Pathophysiology

In persons with pseudolymphoma, lymphocytes and other inflammatory cells are recruited to the skin in response to known or unknown stimuli. Most cases are idiopathic. Cases of cutaneous pseudolymphoma with known etiology include reactions to tattoo dyes, jewelry (especially gold), insect bites, medications,[3] folliculitis, trauma, infections, vaccinations,[4] and contactants. A discrete subset of pseudolymphoma, borrelial lymphocytoma, primarily occurs in Europe in areas endemic for the tick Ixodes ricinus. Borrelial lymphocytoma is a response to infection by Borrelia burgdorferi subsp afzelius conferred by a tick bite. Another subset of pseudolymphoma is the result of an unusual systemic response to medications, typically anticonvulsants (see Drug-Induced Pseudolymphoma Syndrome).

Epidemiology

Frequency

International

No frequency data are available for cutaneous pseudolymphoma; the condition is uncommon but not rare.

Mortality/Morbidity

Pseudolymphoma is not associated with mortality. Localized variants rarely result in morbidity other than minor pain or pruritus. Rare cases of cutaneous pseudolymphoma have been described in which the pseudolymphoma has evolved into cutaneous lymphoma.

Race

Although 90% of reported patients with pseudolymphoma are white, racial predilection has not been established.

Sex

In reported cases of localized pseudolymphoma, the female-to-male ratio is approximately 2:1. No significant epidemiologic data are available regarding entities in the T-cell pattern pseudolymphoma spectrum.

Age

Individuals of any age may be affected, but localized, nodular pseudolymphoma is most common in early life. The mean age of onset is 34 years. Two thirds of patients are younger than 40 years at the time of biopsy. Approximately 8% of cases involve patients younger than 18 years. Borrelial pseudolymphoma is more common in children than in adults.

History

Physical

Causes

Other Tests

Procedures

Histologic Findings

Lymphocytoma cutis must be differentiated from lymphoma. Most examples simulate B-cell lymphoma and show a nodular inflammatory infiltrate in the dermis. The key histologic features that favor pseudolymphoma over lymphoma include the presence of a mixed infiltrate that includes histiocytes, eosinophils, and plasma cells, in addition to lymphocytes, as shown in the image below.[11]


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Biopsy specimens of pseudolymphoma vary substantially, but they most often exhibit a mixed inflammatory infiltrate with prominent lymphoid follicle fo....

The infiltrate in lymphocytoma cutis tends to be more top-heavy, while most lymphomas are centered in the deep dermis or the subcutis. Lymphocytoma cutis typically shows germinal centers and tingible body macrophages. Occasional 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- and T-cell population with a high MIB-1–positive proliferative fraction.[12] Staining for kappa and lambda light chains shows a polyclonal pattern of staining. Fresh, unfixed tissue may be required for adequate assessment of kappa/lambda labeling.

Some cases show a T-cell histologic pattern with a bandlike infiltrate in the papillary dermis, predominantly of small lymphocytes, with variable epidermotropism. Although these features mimic cutaneous T-cell lymphoma/mycosis fungoides, the clinical presentation is often characteristic.[13]

Medical Care

When the offending agent is known, its removal results in resolution of the cutaneous pseudolymphoma. Cases of cutaneous pseudolymphoma documented to occur as a result of infection should be appropriately treated. In idiopathic cases of cutaneous pseudolymphoma, treatment is not mandatory. Cures may be affected via surgical removal, cryosurgery, or local irradiation. Some reports have noted a response to topical or injected corticosteroids and topical immunomodulators such as tacrolimus.

Patients with presumed pseudolymphoma in which the possibility of lymphoma cannot be excluded should be evaluated for the possibility of concurrent extracutaneous disease and followed for possible emergence of lymphoma.

Surgical Care

In cutaneous pseudolymphoma, simple excision of the involved site can be curative in some cases.

Medication Summary

The goals of pharmacotherapy for cutaneous pseudolymphoma are to reduce morbidity and to prevent complications.

Class Summary

These agents have anti-inflammatory properties and cause profound and varied metabolic effects. In addition, these agents modify the body's immune response to diverse stimuli.

Hydrocortisone valerate 0.2% (Westcort)

Clinical Context:  Adrenocorticosteroid derivative suitable for application to skin or external mucous membranes. Has mineralocorticoid and glucocorticoid effects resulting in anti-inflammatory activity. Treats inflammatory dermatosis responsive to steroids. Decreases inflammation by suppressing the migration of polymorphonuclear leukocytes and reversing capillary permeability.

Betamethasone 0.05% cream or ointment (Diprolene, Betatrex)

Clinical Context:  For inflammatory dermatosis responsive to steroids. Decreases inflammation by suppressing migration of polymorphonuclear leukocytes and reversing capillary permeability.

Clobetasol (Temovate)

Clinical Context:  Class I superpotent topical steroid; suppresses mitosis and increases synthesis of proteins that decrease inflammation and cause vasoconstriction.

Fluocinonide cream or ointment 0.05% (Fluonex, Lidex)

Clinical Context:  High-potency topical corticosteroid that inhibits cell proliferation; is immunosuppressive and anti-inflammatory.

Complications

Prognosis

References

  1. Brodell RT, Santa Cruz DJ. Cutaneous pseudolymphomas. Dermatol Clin. Oct 1985;3(4):719-34.[View Abstract]
  2. Choi TS, Doh KS, Kim SH, Jang MS, Suh KS, Kim ST. Clinicopathological and genotypic aspects of anticonvulsant-induced pseudolymphoma syndrome. Br J Dermatol. Apr 2003;148(4):730-6.[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. Maubec E, Pinquier L, Viguier M, et al. Vaccination-induced cutaneous pseudolymphoma. J Am Acad Dermatol. Apr 2005;52(4):623-9.[View Abstract]
  5. Braun RP, French LE, Feldmann R, Chavaz P, Saurat JH. Cutaneous pseudolymphoma, lymphomatoid contact dermatitis type, as an unusual cause of symmetrical upper eyelid nodules. Br J Dermatol. Aug 2000;143(2):411-4.[View Abstract]
  6. Kluger N, Vermeulen C, Moguelet P, et al. Cutaneous lymphoid hyperplasia (pseudolymphoma) in tattoos: a case series of seven patients. J Eur Acad Dermatol Venereol. Feb 2010;24(2):208-13.[View Abstract]
  7. 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]
  8. Michaels B, Michaels J, Mobini N. Prominent lymphoid infiltrate with a pseudolymphoma-like morphology: a new histological finding of injectable liquid silicone. J Cutan Pathol. Nov 2009;36(11):1224-6.[View Abstract]
  9. Gutermuth J, Audring H, Roseeuw D. Disseminated cutaneous B-cell lymphoma mimicking pseudolymphoma over a period of six years. Am J Dermatopathol. Jun 2004;26(3):225-9.[View Abstract]
  10. Braun RP, French LE, Feldmann R, Chavaz P, Saurat JH. Cutaneous pseudolymphoma, lymphomatoid contact dermatitis type, as an unusual cause of symmetrical upper eyelid nodules. Br J Dermatol. Aug 2000;143(2):411-4.[View Abstract]
  11. Burg G, Kerl H, Schmoeckel C. Differentiation between malignant B-cell lymphomas and pseudolymphomas of the skin. J Dermatol Surg Oncol. Apr 1984;10(4):271-5.[View Abstract]
  12. Geerts ML, Kaiserling E. A morphologic study of lymphadenosis benigna cutis. Dermatologica. 1985;170(3):121-7.[View Abstract]
  13. 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]

Pseudolymphomatous drug eruption due to captopril, marked by erythematous to purple papules, patches, and plaques.

This erythrodermic pseudolymphoma (T-cell pattern) typifies drug-induced pseudolymphoma, which is most often secondary to anticonvulsant therapy.

This localized example of pseudolymphoma shows an ill-defined, thin, erythematous plaque.

Biopsy specimens of pseudolymphoma vary substantially, but they most often exhibit a mixed inflammatory infiltrate with prominent lymphoid follicle formation.