Lymphomatoid Papulosis Clinical Presentation

  • Author: John A Zic; Chief Editor: Dirk M Elston, MD   more...
 
Updated: Jan 24, 2012
 

History

  • Most patients with lymphomatoid papulosis (LyP) describe the gradual onset of an asymptomatic to mildly pruritic papular eruption.
    • Papules appear in crops and resolve spontaneously within 2-8 weeks.
    • Waxing and waning of the crops of papules may continue for decades.
  • Unless accompanied by systemic lymphoma, most patients have no constitutional symptoms.
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Physical

  • Unless accompanied by systemic lymphoma, physical findings are limited to the skin and, very rarely, the oral cavity.[8, 9]
  • The primary skin lesions are described as follows:
    • Each erythematous papule evolves into a red-brown, often hemorrhagic, papulovesicular or papulopustular lesion over days to weeks, as demonstrated in the images below. Lymphomatoid papulosis type C on the upper back ofLymphomatoid papulosis type C on the upper back of a 65-year-old woman with waxing and waning papulonodular eruptions for almost 10 years. The eruption was suppressed completely using methotrexate. Lymphomatoid papulosis type A showing a cluster ofLymphomatoid papulosis type A showing a cluster of pink papules and 2 crusted papules that resolved spontaneously in the left axilla of a 68-year-old man. The first symptoms developed in the popliteal fossa 8 years before erupting into more widespread papules 10 months before this photograph was taken.
    • Some lesions develop a necrotic eschar before healing spontaneously. Occasionally, noduloulcerative lesions may be present, as in the image below. Crusted ulcerated papule of lymphomatoid papulosisCrusted ulcerated papule of lymphomatoid papulosis on the left hip of a 47-year-old woman with a longer than 20-year history of recurrent papulonodular eruption with spontaneous resolution.
    • Each papule heals within 2-8 weeks, leaving a hypopigmented or hyperpigmented, depressed, oval, and varioliform scar.
    • Large nodules and plaques may take months to resolve. Carefully evaluate solitary ulcerated nodules, plaques, or masses for CD30+ ALCL (see image below), MF, or rarely, HD. Large indurated plaques of anaplastic large cell lLarge indurated plaques of anaplastic large cell lymphoma of 2-months' duration on the left lateral thigh of a 57-year-old man with a 5-year history of lymphomatoid papulosis. The lymphomatoid papulosis skin lesions (not pictured) were rarely larger than 6 mm.
  • The skin distribution of lesions, characteristically, is on the trunk and extremities, although the palms and/or soles, face, scalp, oral mucosa,[10] and anogenital area also may be involved.
  • Evolving lesions have been described under dermoscopy. The initial papular lesion showed a vascular pattern of tortuous vessels radiating from the center. A white structureless area was seen around the vessels. More mature lesions, hyperkeratotic papules, looked similar except the vascular pattern in the center of the lesion was obscured. As the lesions progressed to necrotic ulcerations, the vascular pattern was only seen at the periphery, while the center of the lesions was a structure of brownish-gray areas. The final, or cicatricial phase, was similar except no vessel pattern was seen.[11]
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Causes

  • The etiology of lymphomatoid papulosis is unknown. Debate persists over whether (1) lymphomatoid papulosis is a benign chronic disorder of activated T cells responding to external or internal stimuli or (2) lymphomatoid papulosis is an indolent T-cell malignancy localized to skin and held in check by the host immune system.
  • A few investigators have discovered viruslike particles in lymphomatoid papulosis lesions examined under electron microscopy.[12]
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Contributor Information and Disclosures
Author

John A Zic  MD, Associate Professor of Medicine/Dermatology, Director, VU Cutaneous Lymphoma Clinic, Vanderbilt University School of Medicine

John A Zic is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, and Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Specialty Editor Board

Gregory J Raugi, MD, PhD  Professor, Department of Internal Medicine, Division of Dermatology, University of Washington at Seattle School of Medicine; Chief, Dermatology Section, Primary and Specialty Care Service, Veterans Administration Medical Center of Seattle

Gregory J Raugi, MD, PhD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Michael J Wells, MD  Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster School of Medicine

Michael J Wells, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, and Texas Medical Association

Disclosure: Nothing to disclose.

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

Daniel S Loo, MD is a member of the following medical societies: American Academy of Dermatology and Association of Professors of Dermatology

Disclosure: Nothing to disclose.

Joel M Gelfand, MD, MSCE  Medical Director, Clinical Studies Unit, Assistant Professor, Department of Dermatology, Associate Scholar, Center for Clinical Epidemiology and Biostatistics, University of Pennsylvania

Joel M Gelfand, MD, MSCE is a member of the following medical societies: Society for Investigative Dermatology

Disclosure: AMGEN Consulting fee Consulting; AMGEN Grant/research funds Investigator; Genentech Grant/research funds investigator; Centocor Consulting fee Consulting; Abbott Grant/research funds investigator; Abbott Consulting fee Consulting; Novartis investigator; Pfizer Grant/research funds investigator; Celgene Consulting fee DMC Chair; NIAMS and NHLBI Grant/research funds investigator

Chief Editor

Dirk M Elston, MD  Director, Ackerman Academy of Dermatopathology, New York

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Additional Contributors

The authors and editors of eMedicine gratefully acknowledge the contributions of previous Chief Editor, William D. James, MD, and previous author, Kristina Collins, MD, to the development and writing of this article.

References

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Lymphomatoid papulosis type C on the upper back of a 65-year-old woman with waxing and waning papulonodular eruptions for almost 10 years. The eruption was suppressed completely using methotrexate.
Crusted ulcerated papule of lymphomatoid papulosis on the left hip of a 47-year-old woman with a longer than 20-year history of recurrent papulonodular eruption with spontaneous resolution.
Large indurated plaques of anaplastic large cell lymphoma of 2-months' duration on the left lateral thigh of a 57-year-old man with a 5-year history of lymphomatoid papulosis. The lymphomatoid papulosis skin lesions (not pictured) were rarely larger than 6 mm.
Lymphomatoid papulosis type A showing a cluster of pink papules and 2 crusted papules that resolved spontaneously in the left axilla of a 68-year-old man. The first symptoms developed in the popliteal fossa 8 years before erupting into more widespread papules 10 months before this photograph was taken.
 
 
 
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