The Experts below are selected from a list of 252 Experts worldwide ranked by ideXlab platform
Bernard A. Cohen - One of the best experts on this subject based on the ideXlab platform.
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pityriasis lichenoides long term follow up study
Pediatric Dermatology, 2018Co-Authors: Julie B Zang, Sarah J Coates, Eric C Vonderheid, Jing Huang, Bernard A. CohenAbstract:BACKGROUND/OBJECTIVES: Pityriasis lichenoides is an uncommon Papulosquamous Disorder of unknown etiology. The objective of this study was to review the clinical features and treatment responses of individuals with pityriasis lichenoides seen at a tertiary referral center. METHODS: Seventy-five patients diagnosed with pityriasis lichenoides between 1997 and 2013 were reviewed, and 46 had long-term follow-up via telephone interviews. RESULTS: Fifty (67%) patients were diagnosed with pityriasis lichenoides chronica, 22 (29%) with pityriasis lichenoides et varioliformis acuta, and 3 (4%) with mixed pityriasis lichenoides chronica and pityriasis lichenoides et varioliformis acuta features. Mean ± standard deviation age at onset was 12 ± 13 years (median 8 years). Disease duration was significantly shorter for patients with pityriasis lichenoides et varioliformis acuta (35 ± 35 months) than for those with pityriasis lichenoides chronica (at least 78 ± 48 months). At long-term follow-up, 23 of 28 (82%) patients with pityriasis lichenoides chronica and 3 of 16 (19%) with pityriasis lichenoides et varioliformis acuta had active disease. None progressed to lymphomatoid papulosis or cutaneous T-cell lymphoma. Ten of 23 active pityriasis lichenoides chronica cases had residual pigmentary change independent of race and lasted at least 35 ± 20 months. The most effective treatments were phototherapy (47% response rate), heliotherapy (33%), topical corticosteroids (27%), and antibiotics (25%). CONCLUSION: Pityriasis lichenoides is a predominantly pediatric Disorder. The time course of pityriasis lichenoides chronica is significantly longer than that of pityriasis lichenoides et varioliformis acuta. Pityriasis lichenoides chronica may persist with pigmentary alterations in the absence of other signs of active inflammation. Treatment response is often limited, particularly for patients with pityriasis lichenoides chronica.
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Pityriasis lichenoides: Long‐term follow‐up study
Pediatric Dermatology, 2018Co-Authors: Julie B Zang, Sarah J Coates, Eric C Vonderheid, Jing Huang, Bernard A. CohenAbstract:Pityriasis lichenoides is an uncommon Papulosquamous Disorder of unknown etiology. The objective of this study was to review the clinical features and treatment responses of individuals with pityriasis lichenoides seen at a tertiary referral center. Seventy-five patients diagnosed with pityriasis lichenoides between 1997 and 2013 were reviewed, and 46 had long-term follow-up via telephone interviews. Fifty (67%) patients were diagnosed with pityriasis lichenoides chronica, 22 (29%) with pityriasis lichenoides et varioliformis acuta, and 3 (4%) with mixed pityriasis lichenoides chronica and pityriasis lichenoides et varioliformis acuta features. Mean ± standard deviation age at onset was 12 ± 13 years (median 8 years). Disease duration was significantly shorter for patients with pityriasis lichenoides et varioliformis acuta (35 ± 35 months) than for those with pityriasis lichenoides chronica (at least 78 ± 48 months). At long-term follow-up, 23 of 28 (82%) patients with pityriasis lichenoides chronica and 3 of 16 (19%) with pityriasis lichenoides et varioliformis acuta had active disease. None progressed to lymphomatoid papulosis or cutaneous T-cell lymphoma. Ten of 23 active pityriasis lichenoides chronica cases had residual pigmentary change independent of race and lasted at least 35 ± 20 months. The most effective treatments were phototherapy (47% response rate), heliotherapy (33%), topical corticosteroids (27%), and antibiotics (25%). Pityriasis lichenoides is a predominantly pediatric Disorder. The time course of pityriasis lichenoides chronica is significantly longer than that of pityriasis lichenoides et varioliformis acuta. Pityriasis lichenoides chronica may persist with pigmentary alterations in the absence of other signs of active inflammation. Treatment response is often limited, particularly for patients with pityriasis lichenoides chronica. © 2018 Wiley Periodicals, Inc.
Frank Burns - One of the best experts on this subject based on the ideXlab platform.
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pityriasis lichenoides a clonal t cell lymphoproliferative Disorder
Human Pathology, 2002Co-Authors: Cynthia M. Magro, Neil A Crowson, A L Kovatich, Frank BurnsAbstract:Pityriasis lichenoides (PL) is a Papulosquamous Disorder often considered a form of reactive dermatosis and classified with small plaque parapsoriasis (digitate dermatosis). However, some patients with PL have developed large plaque parapsoriasis (LPP) and mycosis fungoides (MF), and lymphoid atypia and T-cell clonality have been reported in lesions of PL. We set out to explore the possibility that PL is a form of T-cell dyscrasia. Cases were selected by natural language search from an outpatient dermatopathology database; 35 cases were reviewed and clinicians and patients were contacted. Hematoxylin and eosin–stained sections were examined and immunophenotyping was carried out on paraffin-embedded, formalin-fixed tissue using antibodies to CD2, CD3, CD4, CD5, CD7, CD8, CD20, CD30, and CD56. In paraffin-embedded tissue, T-cell receptor (TCR)- chain rearrangement was sought through polymerase chain reaction single stranded conformational polymorphism analysis. There were 14 males and 21 females with a mean age of 40 years held clinically to have PL chronica (PLC) (28 cases) and/or PL et varioliformis acuta (PLEVA) (7 cases). Five patients developed large atrophic poikilodermatous and/or annular plaques compatible with MF and/or LPP in a background of typical PLC. All biopsies showed tropism of lymphocytes to an epidermis manifesting psoriasiform hyperplasia, dyskeratosis, parakeratosis, and intraepithelial collections of Langerhans’ cells and lymphocytes mimicking Pautrier’s microabascesses. Epidermal atrophy, dermal fibroplasia, poikilodermatous alterations, and a dominance of intraepidermal cerebriform cells were seen only in patients with chronic persistent disease (i.e., PLC) and in some cases corresponded with clinical progression to MF. All cases had a T cell– dominant infiltrate, with a CD7 deletion in 21 of 32 biopsies examined; the CD7- negative cells were typically the largest and most atypical forms, often in a cohesive array within the upper layers of the epidermis. In 17 biopsies in which a CD4 stain was satisfactory for evaluation, 50% or more of the intraepidermal population was CD4 positive in 8 biopsies, whereas in 11 biopsies 50% or more of the dermal infiltrate was CD4 positive. The CD4-positive cells frequently had a cerebriform nuclear morphology and were CD7 negative. Most cases had an admixture of CD8-positive lymphocytes in excess of 40% or more of the intraepidermal and/or dermal infiltrate; it was the dominant intraepidermal infiltrate in 10 cases. The CD8-positive cells, typically small, round, and CD7 positive, showed a directed pattern of migration into acrosyringia and suprapapillary plates, with satellitosis around CD4- positive/CD8-negative/ CD7-negative atypical lymphocytes. CD56 positivity was seen among the intraepidermal lymphoid cells and roughly paralleled the CD8 profile. In general, CD8-positive lymphocytes dominated in cases of PLEVA, whereas CD4-positive lymphocytes were very conspicuous and composed the dominant intraepidermal populace only in those biopsies of progressive PL/PLC. Clonality was shown in 25 of 27 biopsies in which amplifiable DNA was obtained. Intraepithelial atypical lymphocytes, phenotypic abnormalities, and TCR- rearrangements suggest that PLC and PLEVA are a form of T-cell dyscrasia. Lesions may follow a recalcitrant course characteristic of MFand premycotic Disorders such as LPP. The aberrant phenotype cell is similar to that defining MF: a CD4-positive T lymphocyte with a CD5 and CD7 deletion. Directed epidermal migration seen in biopsies procured from incipient lesions along with occasional temporal association to viral or drug exposure suggests that an abnormal immune response to an antigenic trigger may be the inciting event. HUM PATHOL 33:788-795. Copyright 2002, Elsevier Science (USA). All rights reserved. Abbreviations: LPP, large-plaque parapsoriasis; LYP, lymphomatoid papulosis; MF, mycosis fungoides; PL, pityriasis lichenoides; PLC, pityriasis lichenoides chronica; PLEVA, pityriasis lichenoides et varioliformis acuta; PCR, polymerase chain reacrion; TCR, T-cell receptor.
Catherine Smith - One of the best experts on this subject based on the ideXlab platform.
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A systematic review of the literature on the treatment of pityriasis rubra pilaris type 1 with TNF-antagonists
Journal of the European Academy of Dermatology and Venereology : JEADV, 2012Co-Authors: Gabriela Petrof, Noor Almaani, C.b. Archer, W. A. D. Griffiths, Catherine SmithAbstract:Adult pityriasis rubra pilaris (PRP) type 1 is a rare chronic Papulosquamous Disorder with clinical and histological parallels with psoriasis. Treatment is challenging and recent case reports suggest a potential role for tumour necrosis factor (TNF) antagonists. Our objective was to systematically review the literature for evidence of efficacy of TNF antagonists in the treatment of adult PRP. We performed a systematic search of the Cochrane library, EMBASE, Pubmed and MEDLINE databases. We defined diagnosis of PRP, classified clinical response and whether this was clearly attributed to TNF-antagonists. We also reviewed disease, treatment duration and follow up. Sixteen articles were selected for detailed review. From these, 12 articles (13 cases) met our predefined criteria and were included in the systematic review. The authors identified two more cases from their personal archive. A total of 15 evaluable cases were included for analysis. Twelve showed complete response (CR) (80%) to TNF-antagonists with a mean time to maximal response of 5 months. In 10 of the CR cases (83%) this was clearly attributable to TNF antagonist therapy. These data indicate that TNF-antagonists may be of value in treating adult type 1 PRP refractory to other systemic agents but selective reporting bias, together with the lack of standard diagnostic criteria and established spontaneous resolution in PRP, prevent any firm recommendations on their place in management. © 2012 The Authors. Journal of the European Academy of Dermatology and Venereology © 2012 European Academy of Dermatology and Venereology.
Klaus Eisendle - One of the best experts on this subject based on the ideXlab platform.
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Infliximab monotherapy as first-line treatment for adult-onset pityriasis rubra pilaris: case report and review of the literature on biologic therapy.
Journal of the American Academy of Dermatology, 2008Co-Authors: Hansgeorg Müller, Cornelia Gattringer, Bernhard Zelger, Reinhard Höpfl, Klaus EisendleAbstract:Pityriasis rubra pilaris (PRP) is a rare, chronic Papulosquamous Disorder of unknown etiology that often progresses to disabling palmoplantar keratoderma and erythroderma. There is currently no universally effective treatment for PRP, and some cases are resistant to multiple topical and systemic therapies. Systemic retinoids, methotrexate, several immunosuppressive agents, fumaric acid esters, stanozolol, and phototherapy have all been used with varying degrees of success. Recently, a few reports have appeared in the literature concerning the use of biologics in combination therapies and/or in refractory PRP cases. We report a case of type I adult-onset PRP successfully treated with infliximab monotherapy as initial systemic therapy, and provide a comprehensive literature review on biologic therapy for PRP. The complete and persistent response to infliximab in our patient and in the previously reported cases confirms a role for anti-tumor necrosis factor-alfa therapy as an effective option in the treatment of PRP. Further studies are warranted to evaluate possible differences in efficacy among the different biologics.
Diya F. Mutasim - One of the best experts on this subject based on the ideXlab platform.
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Confluent and reticulated papillomatosis without papillomatosis
Journal of the American Academy of Dermatology, 2003Co-Authors: Diya F. MutasimAbstract:Confluent and reticulated papillomatosis is a Papulosquamous Disorder that affects young individuals. There are several hypotheses regarding the cause, including genetic keratinization Disorder, reaction to pityrosporum, and reaction to UV light. Multiple therapeutic agents have been used with variable success. The histologic findings include papillomatosis, hyperkeratosis, and minimal or no acanthosis. We present a patient with the clinical findings of confluent and reticulated papillomatosis who responded dramatically to minocycline, and in whom histologic examination did not reveal papillomatosis.
