Lymphomatoid Papulosis

Clinically benign, histologically malignant, recurrent cutaneous T-cell lymphoproliferative disorder characterized by an infiltration of large atypical cells surrounded by inflammatory cells. The atypical cells resemble REED-STERNBERG CELLS of HODGKIN DISEASE or the malignant cells of CUTANEOUS T-CELL LYMPHOMA. In some cases, lymphomatoid papulosis progresses to lymphomatous conditions including MYCOSIS FUNGOIDES; HODGKIN DISEASE; CUTANEOUS T-CELL LYMPHOMA; or ANAPLASTIC LARGE-CELL LYMPHOMA.

Also Known As:

Networked: 205 relevant articles (11 outcomes, 13 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Lymphoma (Lymphomas)
2.	Parapsoriasis
3.	Skin Diseases (Skin Disease)
4.	Psoriasis (Pustulosis Palmaris et Plantaris)
5.	Atopic Dermatitis (Atopic Eczema)

Experts

1.	Duvic, Madeleine: 7 articles (02/2019 - 01/2003)
2.	Willemze, R: 4 articles (11/2015 - 08/2000)
3.	Kempf, Werner: 4 articles (12/2014 - 08/2004)
4.	Medeiros, L Jeffrey: 4 articles (07/2012 - 08/2004)
5.	Kadin, Marshall E: 3 articles (01/2020 - 08/2004)
6.	Magro, Cynthia M: 3 articles (07/2018 - 04/2006)
7.	Vermeer, M H: 3 articles (11/2015 - 08/2000)
8.	Prince, H Miles: 3 articles (06/2015 - 05/2009)
9.	Aiba, Setsuya: 2 articles (01/2022 - 02/2018)
10.	Fujimura, Taku: 2 articles (01/2022 - 02/2018)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Lymphomatoid Papulosis:

1.	Methotrexate (Mexate)FDA LinkGeneric 07/01/1984 - "Methotrexate appears to be effective in controlling lymphomatoid papulosis and is possibly superior to PUVA in this respect." 06/01/2015 - "The efficacy of methotrexate for lymphomatoid papulosis." 02/01/2022 - "Lymphomatoid papulosis responding to topical methotrexate." 11/01/2011 - "Lymphomatoid papulosis in children: experience of five cases and the treatment efficacy of methotrexate." 01/01/2004 - "The lesions of lymphomatoid papulosis responded to intermittent courses of oral methotrexate. "
2.	Acyclovir (Aciclovir)FDA LinkGeneric 01/01/1986 - "Lymphomatoid papulosis: remission following intravenously administered acyclovir." 10/01/1986 - "[Acyclovir in mycosis fungoides and lymphomatoid papulosis]." 07/11/1986 - "Acyclovir in lymphomatoid papulosis and mycosis fungoides."
3.	Bexarotene (LGD1069)FDA Link 01/01/2003 - "Bexarotene is a new treatment option for lymphomatoid papulosis." 02/01/2018 - "Successful control of phototherapy-resistant lymphomatoid papulosis with oral bexarotene." 04/01/2020 - "Lichen planopilaris in a patient treated with bexarotene for lymphomatoid papulosis."
4.	L-Lactate Dehydrogenase (Lactate Dehydrogenase)IBA 11/01/2010 - "Univariate analysis established that (1) advanced skin and overall clinical stage, increased age, male sex, increased lactate dehydrogenase (LDH), and large-cell transformation were associated with reduced survival and increased RDP; (2) hypopigmented MF, MF with lymphomatoid papulosis, and poikilodermatous MF were associated with improved survival and reduced RDP; and (3) folliculotropic MF was associated with an increased RDP. "
5.	EnzymesIBA 04/01/1983 - "In the present study various immunological, enzyme-histochemical and ultrastructural techniques were used to investigate the cellular infiltrate in both types of lymphomatoid papulosis. " 02/01/1983 - "Based on enzyme cytochemical and immunocytological findings in conjunction with the course of the disease lymphomatoid papulosis is designated as pseudolymphoma of T-cell type." 01/01/1981 - "It was the purpose of this study to further define the nature of the dermal infiltrates in lymphomatoid papulosis (LP) means of enzyme cytochemistry (acid phosphatase and esterase), immunology (rosetting techniques, immunoperoxidase technique on cryostat sections), and by semithin and ultrathin sections. "
6.	DNA (Deoxyribonucleic Acid)IBA 02/01/1994 - "Lymphomatoid papulosis: a clinical and histopathologic review of 53 cases with leukocyte immunophenotyping, DNA flow cytometry, and T-cell receptor gene rearrangement studies." 09/01/1996 - "Fusion of the NPM and ALK genes was detected in three of 18 patients with ALCL who had amplifiable DNA (17%, 95% confidence intervals 4% to 41%), but not in any patients with other NHL, HD, or lymphomatoid papulosis. " 08/01/1989 - "In contrast, analysis by Southern blotting of DNA samples extracted from three nodules from a patient with lymphomatoid papulosis showed no rearranged band and therefore no clonal proliferation. " 09/01/1983 - "DNA analysis by flow cytometry in lymphomatoid papulosis." 07/01/1969 - "DNA measurements in lymphomatoid papulosis: evidence for this new entity."
7.	Brentuximab VedotinIBA 01/01/2021 - "A rare case of early-onset lymphomatoid papulosis refractory to brentuximab vedotin." 09/01/2020 - "Emergence of lymphomatoid papulosis during treatment with brentuximab vedotin." 12/01/2017 - "Brentuximab Vedotin for Patients With Refractory Lymphomatoid Papulosis: An Analysis of Phase 2 Results." 11/10/2015 - "Results of a Phase II Trial of Brentuximab Vedotin for CD30+ Cutaneous T-Cell Lymphoma and Lymphomatoid Papulosis." 11/10/2015 - "Brentuximab vedotin is both active and well tolerated in cutaneous T-cell lymphoma and lymphomatoid papulosis, with an overall response rate of 73% and complete response rate of 35%."
8.	Anaplastic Lymphoma KinaseIBA 04/01/1998 - "Although initially found in primary nodal ALCL, recent studies suggest that NPM-ALK expression may occur in lymphoma at extranodal sites, including the skin; it remains controversial, however, whether CD30+ primary cutaneous lymphoma and its benign counterpart, lymphomatoid papulosis (LyP), express NPM-ALK in some cases. " 11/01/1997 - "Absence of anaplastic lymphoma kinase (ALK) and Epstein-Barr virus gene products in primary cutaneous anaplastic large cell lymphoma and lymphomatoid papulosis." 06/15/1998 - "NPM-ALK transcripts were detected by nested reverse transcription-polymerase chain reaction (RT-PCR) in 1 of 11 lymphomatoid papulosis (LyP), 7 of 15 CD30+ primary cutaneous T-cell lymphoma (CTCL), 3 of 11 CD30+ secondary cutaneous lymphoma, 6 of 27 MF, and 1 of 16 BID. " 07/01/2009 - "CD30 is expressed in various types of cutaneous lymphomas, including lymphomatoid papulosis (LyP), primary cutaneous anaplastic large cell lymphoma (C-ALCL), some cases of mycosis fungoides showing large cell transformation (MF-TR) and skin localizations of systemic anaplastic lymphoma kinase (ALK)-positive or ALK-negative ALCL. " 03/01/2007 - "c-Jun was expressed exclusively by CD30 lymphoproliferative disorders including 41/41 (100%) classical Hodgkin lymphoma (cHL), 20/23 (87%) anaplastic lymphoma kinase (ALK)+ anaplastic large cell lymphoma (ALCL), 18/26 (69%) ALK- ALCL, 5/9 (56%) primary cutaneous ALCL, 4/11 (36%) CD30 diffuse large B-cell lymphoma (DLBCL), and 11/24 (46%) cases of lymphomatoid papulosis. "
9.	AntibodiesIBA 09/01/1976 - "[Lymphomatoid papulosis with presence of anti-vessel antibodies]." 12/01/2014 - "Retrospective case series study with immunohistochemical analysis using anti-CD30 and anti-5-hmC antibodies in control (n = 19), lymphomatoid papulosis (LyP) (n = 27) and primary cutaneous anaplastic large cell lymphoma (ALCL) (n = 14) specimens. " 01/01/1987 - "These data indicate the value of antibodies Ki-1 and Ber-H2 in distinguishing between lymphomatoid papulosis and other types of pre- or pseudo-malignant disorders and support the view that lymphomatoid papulosis, Hodgkin's disease and some types of non-Hodgkin's lymphoma constitute a spectrum of related disorders, originated from activated lymphoid cells." 01/01/1987 - "The antibodies stained the atypical cells in lymphomatoid papulosis, a proportion of the neoplastic cells in some cases of mycosis fungoides and most of the neoplastic cells in six large cell anaplastic/pleomorphic non-Hodgkin's lymphomas. " 10/01/1985 - "Twenty-four biopsy specimens from 14 patients with lymphomatoid papulosis were immunolabeled with antibodies against Ki-1 and other Reed-Sternberg cell-associated antigens. "
10.	Monoclonal AntibodiesIBA 11/01/1985 - "In an attempt to better define the process of lymphomatoid papulosis, two cases were studied by means of light and electron microscopy, immunohistochemistry studies, including the use of monoclonal antibodies, and cytogenetic technics. " 11/01/1985 - "Cryostat sections from fully developed papular lesions of lymphomatoid papulosis (histologic subtype A or B) have been examined by immunoenzymatic staining with 24 monoclonal antibodies against lymphoid cells and their subsets. " 10/01/1985 - "Monoclonal antibody patterns in lymphomatoid papulosis." 08/01/2000 - "Immunostaining with monoclonal antibodies against Fas and Fas-L was performed in material from 23 patients with mycosis fungoides (MF), 10 with lymphomatoid papulosis (LyP), 10 with CD30-positive primary cutaneous large T-cell lymphoma (LTCL) and nine with CD30-negative LTCL. " 11/01/1997 - "To further characterize the cellular origin of the neoplastic T cells, skin biopsies from 14 patients with primary cutaneous CD30-positive (anaplastic) large T-cell lymphomas, nine patients with lymphomatoid papulosis, and six patients with primary cutaneous CD30-negative pleomorphic large T-cell lymphomas were stained with monoclonal antibodies against cytotoxic cell-associated molecules granzyme B and T-cell restricted intracellular antigen. "

Therapies and Procedures

1.	PUVA Therapy 11/01/1982 - "[Lymphomatoid papulosis improved with PUVA therapy]." 08/01/1991 - "A 68-year-old woman affected by mycosis fungoides in the plaque stage noticed that multiple papulonodular lesions of lymphomatoid papulosis developed suddenly after a few sessions of PUVA therapy. " 01/01/2020 - "Methotrexate-resistant acral lymphomatoid papulosis with complete remission after psoralen-ultraviolet A therapy." 03/01/2013 - "Steroid-Resistant Localized Lymphomatoid Papulosis Treated with Local Bath-PUVA Therapy."
2.	Therapeutics 10/01/1992 - "We report on a patient with two nonhealing lymphomatoid papulosis lesions treated successfully with electron beam therapy and describe our technique. " 03/01/2005 - "Medium-dose UVA1 therapy of lymphomatoid papulosis." 02/01/1983 - "121 patients with lymphomatoid papulosis from the literature are reviewed with respect to clinical and histological features, laboratory findings, therapy and course of the disease. " 06/01/2020 - "Lymphomatoid papulosis successfully managed with excimer laser maintenance therapy." 10/01/2000 - "[Therapy of lymphomatoid papulosis with balneo-PUVA-photochemotherapy]]."
3.	Photochemotherapy (Photodynamic Therapy) 09/01/1995 - "PUVA-bath photochemotherapy resulting in rapid clearance of lymphomatoid papulosis in a child." 11/01/2023 - "Refractory lymphomatoid papulosis successfully treated with IFN-α2a and photodynamic therapy." 09/01/2020 - "A case of pediatric lymphomatoid papulosis treated with photodynamic therapy and narrowband ultraviolet B." 01/01/2019 - "Photodynamic therapy can prevent recurrence of lymphomatoid papulosis." 11/01/2009 - "Successful treatment of recalcitrant lymphomatoid papulosis in a child with PUVA-bath photochemotherapy."
4.	Phototherapy (Light Therapy) 06/01/2010 - "Regional lymphomatoid papulosis in a child--treatment with a UVB phototherapy handpiece." 01/01/2004 - "This case illustrates the relapsing and remitting nature of both lymphomatoid papulosis and its potential of developing cutaneous T-cell lymphoma and narrow-band ultraviolet B (NB-UVB) phototherapy as a new modality of treatment of early-stage mycosis fungoides in these patients. " 04/01/1992 - "Phototherapy for the eyelid has not previously been recognized as a safe and effective treatment of photoresponsive dermatoses of the eyelid, such as atopic dermatitis, vitiligo, psoriasis, lymphomatoid papulosis, and parapsoriasis. " 02/01/2018 - "Successful control of phototherapy-resistant lymphomatoid papulosis with oral bexarotene." 09/01/2013 - "We describe the case of a 50-year-old woman diagnosed with lymphomatoid papulosis who was unable to access phototherapy and who failed to clear while on systemic treatment with methotrexate. "
5.	Combination Drug Therapy (Combination Chemotherapy) 11/01/1989 - "The patient's lymphoma responded well to combination chemotherapy, but the lymphomatoid papulosis remains active."