Lymphomatoid papulosis explained

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]

Types of lymphomatoid papulosis^[6]

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

Notes and References

1. Helen H. Wang . Lori Lach . Marshall E. Kadin . Epidemiology of Lymphomatoid Papulosis . The Cancer Journal . 70 . 12 . 2951–2957 . December 1992 . 10.1002/1097-0142(19921215)70:12<2951::aid-cncr2820701236>3.0.co;2-7. 1451079 . 40452892 . free .
2. Macaulay WL . Lymphomatoid papulosis. A continuing self-healing eruption, clinically benign--histologically malignant . Arch Dermatol . 97 . 1 . 23–30 . January 1968 . 5634442 . 10.1001/archderm.97.1.23.
3. El Shabrawi-Caelen L, Kerl H, Cerroni L . Lymphomatoid papulosis: reappraisal of clinicopathologic presentation and classification into subtypes A, B, and C . Arch Dermatol . 140 . 4 . 441–7 . April 2004 . 15096372 . 10.1001/archderm.140.4.441 . https://archive.today/20110524093906/http://archderm.ama-assn.org/cgi/pmidlookup?view=long&pmid=15096372 . 2011-05-24 .
4. Book: Neal S. Young. Stanton L. Gerson. Katherine A. High. Clinical hematology. 14 May 2011. 2006. Elsevier Health Sciences. 978-0-323-01908-8. 555–.
5. Book: Maria Proytcheva. Diagnostic Pediatric Hematopathology. 15 May 2011. 14 March 2011. Cambridge University Press. 978-0-521-88160-9. 544–.
6. Hughey LC. Practical Management of CD30⁺ Lymphoproliferative Disorders. . Dermatol Clin . 2015 . 33 . 4 . 819–33 . 26433852 . 10.1016/j.det.2015.05.013 . free .
7. Magro CM, Crowson AN, Morrison C, Merati K, Porcu P, Wright ED . CD8+ lymphomatoid papulosis and its differential diagnosis . . 125 . 4 . 490–501 . April 2006 . 16627259 . 10.1309/NNV4-L5G5-A0KF-1T06 . free .
8. Dalle S, Balme B, Thomas L . Lymphomatoid papulosis localized to the face . Dermatol. Online J. . 12 . 3 . 9 . 2006 . 10.5070/D35DS430MG . 16638423 .