Lymphomatoid papulosis in Child
Alerts and Notices
Synopsis
LyP has an incidence of roughly 1.2-1.9 cases per 1 000 000 people and may occur at any age, although it is unusual in childhood. LyP is characterized by recurrent crops of pruritic, papulonecrotic, or nodular lesions that are self-healing over weeks or months. Lesions in various stages of healing are often seen. Lesions are often pruritic and may resolve with scarring. Six histopathologic subtypes are recognized by the 2018 World Health Organization-European Organization for Research and Treatment of Cancer (WHO-EORTC) classification and are described in Best Tests. Specific subtypes of LyP do not correlate with clinical findings. Both the clinical history and histopathologic findings must be consistent with LyP for diagnosis; histopathology alone is not sufficient.
The cause of LyP is unknown, and its pathogenesis is only partially understood. Interactions between CD30 and its ligand appear to be important, and mutations in TGF-beta receptors may contribute. Identification of human T-cell lymphotropic virus type 1 (HTLV-1), herpesviruses 6, 7, and 8, and human endogenous retroviruses within LyP has been reported, but no clear relationship has been found. Several reports also suggest radiation therapy and fingolimod as triggers.
There is debate whether LyP is an inflammatory disorder or an indolent T-cell malignancy, but most authors categorize it as the latter. Most patients with LyP have chronic, self-limited eruptions. It is estimated that 15%-50% of patients with LyP will develop another lymphoproliferative disorder, such as ALCL, Hodgkin disease, or mycosis fungoides (MF). Patients with LyP should be monitored closely for these additional malignancies. A cohort study comprised of 504 LyP patients found that there was an increased risk of developing nonhematologic malignancies such as squamous cell carcinoma (SCC), melanoma, bladder cancer, and lung cancer. Further data confirming these findings are needed.
LyP has a 10-year disease-specific survival rate of close to 100%.
Codes
ICD10CM:C86.6 – Primary cutaneous CD30-positive T-cell proliferations
SNOMEDCT:
31047003 – Lymphomatoid papulosis
Look For
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Diagnostic Pearls
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Differential Diagnosis & Pitfalls
- Pityriasis lichenoides et varioliformis acuta (PLEVA) – more common than LyP in childhood
- Other forms of cutaneous lymphoma
- Lymphomatoid drug reactions (see pseudolymphoma)
- Folliculitis
- Insect bites
- Langerhans cell histiocytosis
- Pseudolymphoma
- Scabies
- Rickettsialpox
- Disseminated herpes simplex
- Papulonecrotic tuberculid (see cutaneous tuberculosis)
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.Subscription Required
References
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Last Reviewed:08/07/2022
Last Updated:08/08/2022
Last Updated:08/08/2022
Lymphomatoid papulosis in Child