Lymphomatoid Papulosis Clinical Presentation

Updated: Oct 29, 2018
  • Author: John A Zic, MD; Chief Editor: William D James, MD  more...
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Presentation

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 Examination

Unless accompanied by systemic lymphoma, physical findings are limited to the skin and, very rarely, the oral cavity. [18, 19]

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 of 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.
Lymphomatoid papulosis type A showing a cluster of 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.

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 papulosis 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.

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 l 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.

The skin distribution of lesions, characteristically, is on the trunk and extremities, although the palms and/or soles, face, scalp, oral mucosa, [20] 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. [21]

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