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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]

Immune System

  • 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]

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

Review 11 months post-photodynamic therapy demonstrated complete clinical clearance at the treatment site. [ncbi.nlm.nih.gov]

Lymphomatoid Papulosis Treatment Tips 1. Cortisone ointments will sometimes help. 2. Ultraviolet light treatment will help control lymphomatoid papulosis. 3. Methotrexate is sometimes used to treat lymphomatoid papulosis. 4. [streetdirectory.com]

Treatment of lymphomatoid papulosis mainly focusses on reducing the symptoms and timely detection of early lymphoma. [epainassist.com]

Treatment does not guarantee total regression of lesions and relapses are common with the use of lower doses or the discontinuation of treatment. Interferon alfa-2a can be used as an alternative to MTX treatment. [orpha.net]

Prognosis

What is the Prognosis of Lymphomatoid Papulosis? (Outcomes/Resolutions) The prognosis of Lymphomatoid Papulosis in a majority of individuals is excellent, since LyP is a self-limiting condition that resolves on its own. [dovemed.com]

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]

Etiology

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]

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 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]

It is a rare recurrent chronic skin disease, and of unknown etiology. PL is included in the TLTCP group, along with anaplastic large cell cutaneous lymphoma and borderline lesions. [dialnet.unirioja.es]

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]

Beth Israel Deaconess Medical Center Research Abstract My research has been in three general areas: epidemiology of chronic diseases, gastrointestinal pathology, and cytopathology. [dfhcc.harvard.edu]

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]

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]

Our results provide new insights on MC distribution in CTCL reappraising their role in the pathophysiology of CTCL. [journals.plos.org]

Prevention

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]

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]

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]

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