Lymphomatoid papulosis
| Lymphomatoid papulosis | |
|---|---|
| Specialty | Dermatology  |
Lymphomatoid papulosis (LyP) is a rare
skin disorder
.
Prevalence
The overall prevalence rate of lymphomatoid papulosis is estimated at at least 1.2 cases per 1,000,000 population.[1] This rare condition has only been studied in depth since 1968.[2]
Presentation
It can appear very similar to
anaplastic large cell lymphoma.[3]
Type "A" is CD30 positive, while type "B" is CD30 negative.[4]
It has been described as "clinically benign but histologically malignant."[5]
| A | Wedge-shaped clusters of large atypical lymphocytes that are CD30+, interspersed with a mixed inflammatory infiltrate of neutrophils, histiocytes, and eosinophils. |
|---|---|
| B | Similar to mycosis fungoides, with bandlike infiltrate and epidermotropism of smaller atypical lymphocytes that may be CD30-. |
| C | Similar to cutaneous anaplastic large-cell lymphoma, with larger clusters or sheets of large anaplastic CD30+ cells without the interspersed mixed infiltrate of Type A. |
| D | Similar to CD8+ epidermotropic cutaneous T-cell lymphoma, with large CD8+ and CD30+ lymphocytes that often stain with cytotoxic markers (TIA-1, granzyme, perforin). |
| E | Angioinvasive with small to large angiocentric CD30+ atypical lymphocytes that invade walls of small to medium vessels in dermis or subcutaneously. |
| F | Perifollicular infiltrates of CD30+ atypical cells with folliculotrophism with or without follicular mucinosis. |
Treatment
It may respond to methotrexate or PUVA.[7]
Prognosis
It can evolve into lymphoma.[8]
See also
- Cutaneous T-cell lymphoma
- Parapsoriasis
- Secondary cutaneous CD30+ large cell lymphoma
- List of cutaneous conditions
References
- S2CID 40452892.
- PMID 5634442.
- PMID 15096372. Archived from the originalon 2011-05-24.
- ISBN 978-0-323-01908-8. Retrieved 14 May 2011.
- ISBN 978-0-521-88160-9. Retrieved 15 May 2011.
- PMID 26433852.
- PMID 16627259.
- PMID 16638423.