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Lymphomatoid papulosis in Adult
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Lymphomatoid papulosis in Adult

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Contributors: Noah Craft MD, PhD, Belinda Tan MD, PhD, Whitney A. High MD, JD, MEng
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Synopsis

Lymphomatoid papulosis (LyP) is a recurrent, chronic, self-healing eruption that exists on a continuum with other CD30-positive cutaneous lymphoproliferative disorders. It was recognized as a distinct disease entity because of its juxtaposed malignant histopathologic qualities but generally benign clinical course.

The cause of LyP is unknown. There is debate as to whether LyP is an inflammatory disorder or an indolent T-cell malignancy. Most patients with LyP have chronic, self-limited eruptions. It is estimated that 10%-20% of patients with LyP will develop another lymphoproliferative disorder, such as CD30-positive anaplastic large cell lymphoma (ALCL), Hodgkin disease, or cutaneous T-cell lymphoma (CTCL; mycosis fungoides [MF]). Evidence has demonstrated clonal similarities between the cells of LyP and the cells of CTCL in patients with both diseases, suggesting a common origin.

LyP is characterized clinically by recurrent crops of pruritic, papulonecrotic papules. Five recognized histopathologic subtypes (A, B, C, D, and E) currently exist, and mixed patterns may be observed. A new subtype with characteristic chromosomal aberrations has been postulated. Clinical features of LyP do not correlate with the histopathologic subtype, and this is central to assessment.

LyP may occur at any age, although it is unusual in childhood. Lesions are often pruritic and may resolve with scarring. Patients should be followed for possible development of another lymphoproliferative disorder.

Codes

ICD10CM:
D23.9 – Other benign neoplasm of skin, unspecified

SNOMEDCT:
31047003 – Lymphomatoid papulosis

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

Pityriasis lichenoides et varioliformis acuta (PLEVA) is more common than LyP in childhood, but it is not associated with malignant disease. PLEVA is also not often characterized by the CD30-positive lymphocytes that are a typical feature of LyP.

Best Tests

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Management Pearls

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Therapy

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Drug Reaction Data

Below is a list of drugs with literature evidence indicating an adverse association with this diagnosis. The list is continually updated through ongoing research and new medication approvals. Click on Citations to sort by number of citations or click on Medication to sort the medications alphabetically.

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References

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Last Updated: 08/09/2019
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Lymphomatoid papulosis in Adult
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Lymphomatoid papulosis : Scattered many, Eschars, Firm papules
Clinical image of Lymphomatoid papulosis
Numerous erythematous papules and some nodules, one with a central crusted ulcer, on the arm.
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