Lymphomatoid papulosis

Lymphomatoid papulosis
SpecialtyDermatology

Lymphomatoid papulosis (LyP) is a rare skin disorder.[1] 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 [2]]. This rare condition has only been studied in depth since 1968.[3]

Presentation

It can appear very similar to anaplastic large cell lymphoma.[4] Type "A" is CD30 positive, while type "B" is CD30 negative.[5]

It has been described as "clinically benign but histologically malignant."[6]

  • Lymphomatoid papulosis
    Lymphomatoid papulosis
  • Lymphomatoid papulosis
    Lymphomatoid papulosis
  • Lymphomatoid papulosis type E
    Lymphomatoid papulosis type E

Cause

The cause of this condition is not established at this time, however stress or anxiety may be a risk factor[7]

Diagnosis

Diagnosis of lymphomatoid papulosis is done via the presentation and a skin biopsy[7]

Treatment

In terms of management, lymphomatoid papulosis may respond to methotrexate or PUVA.[8]

Prognosis

It can evolve into lymphoma,[9] though the mortality rate is low[7]

Additional images

  • Lymphomatoid papulosis/pathology
    Lymphomatoid papulosis/pathology
  • Lymphomatoid papulosis/pathology
    Lymphomatoid papulosis/pathology
  • Lymphomatoid papulosis/pathology
    Lymphomatoid papulosis/pathology

See also

References

  1. ↑ Toumi, Asma; Fazal, Salman; Litaiem, Noureddine (2021). "Lymphomatoid Papulosis". StatPearls. StatPearls Publishing. Archived from the original on 28 August 2021. Retrieved 22 May 2021.
  2. ↑ 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.{{cite journal}}: CS1 maint: multiple names: authors list (link)
  3. ↑ 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.
  4. ↑ 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.
  5. ↑ 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.
  6. ↑ Maria Proytcheva (14 March 2011). Diagnostic Pediatric Hematopathology. Cambridge University Press. pp. 544–. ISBN 978-0-521-88160-9. Retrieved 15 May 2011.
  7. 1 2 3 "Lymphomatoid papulosis | Genetic and Rare Diseases Information Center (GARD) – an NCATS Program". rarediseases.info.nih.gov. Archived from the original on 18 March 2021. Retrieved 22 May 2021.
  8. ↑ 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.
  9. ↑ Dalle S, Balme B, Thomas L (2006). "Lymphomatoid papulosis localized to the face". Dermatol. Online J. 12 (3): 9. PMID 16638423. Archived from the original on 2012-10-01. Retrieved 2021-04-06.
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