Edit concept Question Editor Create issue ticket

Lymphomatoid Papulosis

LyP


Presentation

  • To report an unusual presentation of a case of Lymphomatoid papulosis(LyP) in a young girl. A 14-year-old female presented with a history of swelling of the left upper eyelid of two weeks duration.[ncbi.nlm.nih.gov]
  • We report a 13-year-old girl who presented with typical LyP and pathological features of subtype A. Six months later, the patient presented with rapidly progressive peripheral and systemic lymphadenopathy.[ncbi.nlm.nih.gov]
  • The remarkable cutaneous necrosis showed in our case might be related to the angiodestructive infiltrate that was present in this circumstance.[ncbi.nlm.nih.gov]
  • METHODS: A 74-year-old man with a 3-year history of LP presented with oliguria. He was found to have acute renal failure and 37 g of proteinuria.[ncbi.nlm.nih.gov]
  • Of these patients, 78 presented only 1 histopathologic subtype of lymphomatoid papulosis (64 had type A, 3 had type B, and 11 had type C). The last 7 patients presented more than 1 subtype (1 had A and B, 5 had A and C, and 1 had A, B, and C).[ncbi.nlm.nih.gov]
Constitutional Symptom
  • Unless accompanied by systemic lymphoma, most patients have no constitutional symptoms, and physical findings are limited to the skin.[ncbi.nlm.nih.gov]
  • Clinical evaluation should include a complete history assessing for atypical features, including previous lymphoid neoplasms, constitutional symptoms, lymphadenopathy, or laboratory abnormalities on complete blood counts, blood chemistry, and lactate[clinicalgate.com]
Abdominal Lymphadenopathy
  • Chest CT-Scan did not find mediastinal or abdominal lymphadenopathy. Histopathological examination of a skin biopsy taken from a cheek lesion revealed an epidermal hyperplasia and a dense infiltrate in the dermis with epidermotropism.[dermatology.cdlib.org]
  • Radiologic examination of chest revealed fibrostreaky densities in right upper lobe and CT scan of abdomen showed no intra-abdominal lymphadenopathy.[kjim.org]
Withdrawn
  • The drug was withdrawn. Resolution began only 2 days later. Lymphomatoid papulosis is a benign subtype of cutaneous T-cell lymphoma, but up to 20% of cases can transform to a malignant course.[ncbi.nlm.nih.gov]
  • LYP was unrelated to cyclosporin (second episode after its withdrawn) but preceded the recurrence of proteinuria, suggesting a relationship with the disease activity.[ncbi.nlm.nih.gov]
Dizziness
  • Five years later he presented with an acute onset of nausea and vomiting, dizziness, headache and ataxia. Magnetic resonance imaging of the brain revealed a lesion in the cerebellum and stereotactic resection was undertaken.[ncbi.nlm.nih.gov]
  • Adverse events of grade 3 or higher were neutropenia (n   2) and dizziness/vertigo (n   1). Three patients withdrew owing to adverse events.[ncbi.nlm.nih.gov]
Vertigo
  • Adverse events of grade 3 or higher were neutropenia (n   2) and dizziness/vertigo (n   1). Three patients withdrew owing to adverse events.[ncbi.nlm.nih.gov]
Oliguria
  • METHODS: A 74-year-old man with a 3-year history of LP presented with oliguria. He was found to have acute renal failure and 37 g of proteinuria.[ncbi.nlm.nih.gov]

Workup

  • We recommend that a search for thymoma be included in the workup of LyP. Further chemokine profiling in other cases of LyP may assist in understanding their role in this disease.[ncbi.nlm.nih.gov]
  • Histologic examination is the first diagnostic step in the diagnostic workup of clinically suspected CD30 LPDs.[bloodjournal.org]

Treatment

  • Treatment was started using a UVB phototherapy handpiece (twice weekly), with resolution of the lesions after 6 weeks of treatment. There was a relapse after 9 months with a good response after six more sessions of treatment.[ncbi.nlm.nih.gov]
  • Review 11 months post-photodynamic therapy demonstrated complete clinical clearance at the treatment site.[ncbi.nlm.nih.gov]
  • It represents a potential treatment for the CD30 lymphoproliferative disorder lymphomatoid papulosis (LyP), which currently has no approved treatment. Objective: To assess the efficacy and safety of brentuximab vedotin for the treatment of LyP.[ncbi.nlm.nih.gov]
  • Treatment was initiated for a primary central nervous system (CNS) lymphoma but ceased after one course of high-dose methotrexate.[ncbi.nlm.nih.gov]
  • OBJECTIVE: The aim of this study was to evaluate characteristics, risk factors, associated malignancies, long-term outcome, and treatment of LyP in a large cohort representing the experience of the MD Anderson Cancer Center.[ncbi.nlm.nih.gov]

Prognosis

  • IMPLICATIONS FOR PRACTICE: The management of lymphomatoid papulosis (LyP) is that of an indolent cutaneous lymphoma, based on its excellent prognosis. However, this good prognosis is altered if LyP is associated with lymphoma.[ncbi.nlm.nih.gov]
  • Abstract Fip1-like 1/platelet-derived growth factor receptor-alpha (FIP1L1/PDGFRA)-positive hypereosinophilic syndrome is a rare disorder with a poor prognosis if untreated and for which treatment with imatinib mesilate is highly effective.[ncbi.nlm.nih.gov]
  • Expression of CD56 is associated with a poor prognosis in subcutaneous panniculitis-like T-cell lymphoma and blastic natural killer cell lymphoma.[ncbi.nlm.nih.gov]
  • Lymphomatoid papulosis (LyP) is an uncommon CD30 lymphoproliferative disorder with a relatively excellent prognosis.[ncbi.nlm.nih.gov]
  • Given the excellent long-term prognosis, recent consensus guidelines indicate that patients can be monitored off therapy.[ncbi.nlm.nih.gov]

Etiology

  • Even though the etiology of LYP is, in this case, uncertain, it should be considered as a clinical association of nephrotic syndrome in children and also included among potential triggers of the disease.[ncbi.nlm.nih.gov]
  • Etiology Etiology is unknown. In some reports, a correlation has been found between the use of immunosuppressive medication (e.g. anti-tumor necrosis factor (TNF) agents) for the treatment of chronic inflammatory diseases and the occurrence of LyP.[orpha.net]
  • Etiology No etiologic factors are known. Epidemiology All reported cases occurred in elderly adults (67-88 years, mean 75 years) with strong male predominance (9M:2F) (Karai et al., 2013).[atlasgeneticsoncology.org]
  • ETIOLOGY Lymphomatoid papulosis is characterized by chronic, recurrent and self-limited papulonodular skin eruptions.1 It may occur at any age but is most frequently seen in 20- to 40-year-old adults. Systemic symptoms are typically minimal.[the-dermatologist.com]
  • Although the etiology of localized LyP remains unknown, considering that 2 of 5 reported patients developed widespread lesions regional LyP may be the initial presentation of typical LyP [ 2, 7, 6, 9 ].[dermatology.cdlib.org]

Epidemiology

  • AIM: To analyse a cohort of Asian patients with LyP, diagnosed from 1987 to 2007 at the National Skin Centre (NSC), Singapore, in terms of epidemiology, treatment and association with a second lymphoma.[ncbi.nlm.nih.gov]
  • Summary Epidemiology Exact prevalence is unknown.[orpha.net]
  • […] primary cutaneous anaplastic large cell lymphoma [C-ALCL], and borderline cases) ETIOLOGY/PATHOGENESIS Idiopathic Viral infection, chronic antigenic stimulation, and immunosuppression have all been implicated as factors in some cases CLINICAL ISSUES Epidemiology[basicmedicalkey.com]
  • Overall estimated median disease-free survival (DFS) was 11 months (95% CI 9-13 months) but DFS for patients treated with phototherapy was 23 months (95% CI 10-36 months; P CONCLUSIONS: Current epidemiological, clinical and pathological data support previous[ncbi.nlm.nih.gov]
  • Epidemiology of lymphomatoid papulosis. Cancer. 1992 Dec 15. 70(12):2951-7. [Medline]. Moura FN, Thomas L, Balme B, Dalle S. Dermoscopy of lymphomatoid papulosis. Arch Dermatol. 2009 Aug. 145(8):966-7. [Medline]. Vonderheid EC, Kadin ME, Gocke CD.[emedicine.com]
Sex distribution
Age distribution

Pathophysiology

  • The pathophysiology of CD30 LPDs, including lymphomatoid papulosis (LyP), is largely unknown.[emedicine.com]
  • JAMA (1986) [ Pubmed ] CD30-CD30 ligand interaction in primary cutaneous CD30( ) T-cell lymphomas: A clue to the pathophysiology of clinical regression.[wikigenes.org]
  • CD30-CD30 ligand interaction in primary cutaneous CD30 T-cell lymphomas: a clue to the pathophysiology of clinical regression Blood. 1999;943077- 3083 PubMed Google Scholar 33. Beljaards RCKaudewitz PBerti E et al.[jamanetwork.com]

Prevention

  • Treatment provided symptomatic relief but did not prevent progression to lymphoma. LIMITATIONS: The limitation of this study is the retrospective study design. CONCLUSION: Patients with LyP are at increased risk of associated lymphomas.[ncbi.nlm.nih.gov]
  • By following a healthy diet and exercise, symptoms of lymphomatoid papulosis can be prevented therefore check-ups are mostly the best preventive measures you can take.[epainassist.com]
  • Steroid creams do not prevent the development of new lesions but may be helpful in reducing the lesion symptoms. Cortisone treatments have been suggested to help heal the condition.[streetdirectory.com]
  • Topical bexarotene, in a region of localized disease, may prevent recurrence of the condition. Similarly, topical imiquimod cream 5% can hasten resolution of lesions.[clinicalgate.com]

Ask Question

5000 Characters left Format the text using: # Heading, **bold**, _italic_. HTML code is not allowed.
By publishing this question you agree to the TOS and Privacy policy.
• Use a precise title for your question.
• Ask a specific question and provide age, sex, symptoms, type and duration of treatment.
• Respect your own and other people's privacy, never post full names or contact information.
• Inappropriate questions will be deleted.
• In urgent cases contact a physician, visit a hospital or call an emergency service!