Lymphomatoid papulosis

Lymphomatoid papulosis (LyP) is a rare skin disorder. The overall prevalence rate of lymphomatoid papulosis is estimated at 1.2 to 1.9 cases per 1,000,000 population. [This is a widespread misinterpretation of a 1992 study saying "the period prevalence rate of lymphomatoid papulosis was estimated to be 1.9 per 1,000,000 population for Massachusetts and 1.2 per 1,000,000 population for Pennsylvania". The authors of that study said clearly "Our estimate of 1.2-1.9 cases per 1,000,000 population should be considered a minimum estimate of the prevalence rate". That estimate was based on the 78 patients involved in the study, not the LvP population. The study recruited 11 patients from Massachusetts and 15 from Pennsylvania [1]]. This rare condition has only been studied in depth since 1968.[2]

Lymphomatoid papulosis
SpecialtyDermatology 

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]

Treatment

It may respond to methotrexate or PUVA.[6]

Prognosis

It can evolve into lymphoma.[7]

See also

References

  1. 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.{{cite journal}}: CS1 maint: multiple names: authors list (link)
  2. 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.
  3. 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.
  4. 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.
  5. Maria Proytcheva (14 March 2011). Diagnostic Pediatric Hematopathology. Cambridge University Press. pp. 544–. ISBN 978-0-521-88160-9. Retrieved 15 May 2011.
  6. Magro CM, Crowson AN, Morrison C, Merati K, Porcu P, Wright ED (April 2006). "CD8+ lymphomatoid papulosis and its differential diagnosis". Am. J. Clin. Pathol. 125 (4): 490–501. doi:10.1309/NNV4-L5G5-A0KF-1T06. PMID 16627259.
  7. 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.
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