Lymphomatoid papulosis (LyP) is a rare skin disorder.
| Lymphomatoid papulosis | |
|---|---|
| Specialty | Dermatology  |
Prevalence
editThe 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
editIt 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
editIt may respond to methotrexate or PUVA.[7]
Prognosis
editSee also
editReferences
edit- ^ Helen H. Wang; Lori Lach; Marshall E. Kadin (December 1992). "Epidemiology of Lymphomatoid Papulosis". The Cancer Journal. 70 (12): 2951–2957. doi:10.1002/1097-0142(19921215)70:12<2951::aid-cncr2820701236>3.0.co;2-7. PMID 1451079. S2CID 40452892.
- ^ Macaulay WL (January 1968). "Lymphomatoid papulosis. A continuing self-healing eruption, clinically benign--histologically malignant". Arch Dermatol. 97 (1): 23–30. doi:10.1001/archderm.97.1.23. PMID 5634442.
- ^ El Shabrawi-Caelen L, Kerl H, Cerroni L (April 2004). "Lymphomatoid papulosis: reappraisal of clinicopathologic presentation and classification into subtypes A, B, and C". Arch Dermatol. 140 (4): 441–7. doi:10.1001/archderm.140.4.441. PMID 15096372. Archived from the original on 2011-05-24.
- ^ Neal S. Young; Stanton L. Gerson; Katherine A. High (2006). Clinical hematology. Elsevier Health Sciences. pp. 555–. ISBN 978-0-323-01908-8. Retrieved 14 May 2011.
- ^ Maria Proytcheva (14 March 2011). Diagnostic Pediatric Hematopathology. Cambridge University Press. pp. 544–. ISBN 978-0-521-88160-9. Retrieved 15 May 2011.
- ^ Hughey LC (2015). "Practical Management of CD30⁺ Lymphoproliferative Disorders". Dermatol Clin. 33 (4): 819–33. doi:10.1016/j.det.2015.05.013. PMID 26433852.
- ^ Magro CM, Crowson AN, Morrison C, Merati K, Porcu P, Wright ED (April 2006). "CD8+ lymphomatoid papulosis and its differential diagnosis". American Journal of Clinical Pathology. 125 (4): 490–501. doi:10.1309/NNV4-L5G5-A0KF-1T06. PMID 16627259.
- ^ Dalle S, Balme B, Thomas L (2006). "Lymphomatoid papulosis localized to the face". Dermatol. Online J. 12 (3): 9. doi:10.5070/D35DS430MG. PMID 16638423.