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The British Journal of Dermatology Dec 2020Pityriasis lichenoides (PL) is a papulosquamous dermatosis affecting both children and adults, for which no standard treatment currently exists.
BACKGROUND
Pityriasis lichenoides (PL) is a papulosquamous dermatosis affecting both children and adults, for which no standard treatment currently exists.
OBJECTIVES
To characterize different treatment options and develop an evidence-based treatment algorithm for PL.
METHODS
A systematic search of published literature on PL treatments was performed on 23 December 2017 via the MEDLINE, Embase, CINAHL, CENTRAL, ClinicalTrials.gov and the EU Clinical Trials Register databases.
RESULTS
Of 1090 abstracts retrieved, 27 full-text articles with 502 participants were included for analysis. Seventeen of the full-text articles were retrospective cohort studies and two were randomized controlled studies. Treatment modalities included in these articles were phototherapy, antibiotics, methotrexate, pyrimethamine and trisulfapyrimidine, corticosteroids and conservative treatment. Of these treatments, phototherapy led to complete remission in the highest proportion of patients, and topical corticosteroids were found to have been trialled in the highest number of patients.
CONCLUSIONS
The current literature consists almost entirely of uncontrolled studies, and none provides compelling data to support an evidence-based approach to PL treatment. Pityriasis lichenoides chronica and pityriasis lichenoides et varioliformis acuta should be distinguished in response to treatment, and definitions of response to treatment must be standardized. Additional randomized control studies with longer follow-up will help better differentiate between treatment efficacies and adverse effects.
Topics: Adrenal Cortex Hormones; Adult; Anti-Bacterial Agents; Child; Humans; Phototherapy; Pityriasis Lichenoides; Retrospective Studies
PubMed: 32112390
DOI: 10.1111/bjd.18977 -
Qatar Medical Journal 2023Acute urticaria is urticaria with or without angioedema that is present for less than six weeks, while chronic urticaria is present for more than six weeks. Pityriasis...
BACKGROUND
Acute urticaria is urticaria with or without angioedema that is present for less than six weeks, while chronic urticaria is present for more than six weeks. Pityriasis lichenoides (PL) is a benign cutaneous inflammatory disease of unknown etiology. Acute PL typically resolves within a few weeks, while chronic PL lasts several months. The skin rash of PL may resemble the rash of other conditions, so the distinction is essential and depends on history and physical examination and is confirmed by skin biopsy.
CASE REPORT
A 64-year-old gentleman presented with seven days history of generalized itchy skin (hives). Individual lesions last 24-48 hours and do not leave pigmentation or scarring. No systemic involvement. No specific triggers, with two previous similar episodes 30 years and 20 years ago. Levocetirizine, 5 mg tablet, was prescribed, and he was instructed to increase the dose to 4 tablets daily if needed. On reassessing the patient after ten days, he did not respond well. The rash was different from the initial one, with individual lesions lasting for five days or more, so he was referred to a dermatologist for a skin biopsy. Basic investigations were normal. Performing skin biopsy is needed to exclude other pathologies. Skin biopsy showed pathological changes of lichenoid dermatitis compatible with pityriasis lichenoides et varioliformis acuta (PLEVA). He has been treated with azithromycin 250 mg daily for three weeks with rapid and complete resolution without scaring.
CONCLUSION
Urticarial rash may mimic the skin rash of other conditions. Detailed serial history and physical examination are warranted to exclude other diagnoses. Skin biopsy is needed when diagnosing conditions other than urticaria are suspected.
PubMed: 38025325
DOI: 10.5339/qmj.2023.sqac.7 -
Archives of Dermatological Research Mar 2023Mycosis fungoides (MF) is the most common subtype of primary cutaneous T cell lymphomas, whereas pityriasis lichenoides chronica (PLC) is a chronic inflammatory skin...
Mycosis fungoides (MF) is the most common subtype of primary cutaneous T cell lymphomas, whereas pityriasis lichenoides chronica (PLC) is a chronic inflammatory skin disorder. The inflammasome is a part of the natural immune system which has a multimeric structure consisting of the receptor, adaptor and effector protein that show specificity for various ligands or activators. After the activation of the inflammasome complex, caspase 1 becomes activated which subsequently triggers interleukin-18 (IL-18) and interleukin-1β (IL-1β) production. In our study we aimed to examine the roles of nucleotide-binding oligomerization domain-like receptor containing pyrin domain 1 (NLRP1) and nucleotide-binding oligomerization domain-like receptor containing pyrin domain (NLRP3) inflammasomes in the etiopathogeneses of PLC and MF. NLRP1, NLRP3, caspase 1, IL-18 and IL-1β levels were examined and compared immunohistochemically in the skin biopsies belonging to 16 control patients; 16 PLC cases, 12 cases with stage 1 MF and 12 cases with other stages of MF (stage 2-4). In the paired comparisons of NLRP1, stage 2-4 MF group and PLC group were shown to have increased levels of NLRP1 expression compared to the control group. IL-1β was also expressed at statistically significantly higher levels in each of the stage 1 MF, stage 2-4 MF and PLC groups compared to the control group. In the paired comparisons of caspase 1 and IL-18, it was found that stage 1 MF, stage 2-4 MF and PLC groups had increased levels of expression compared to the control group. Our findings suggest that the NLRP1 inflammasome pathway might play a role in the etiopathogenesis and progression of PLC and MF.
Topics: Humans; Inflammasomes; NLR Family, Pyrin Domain-Containing 3 Protein; Interleukin-18; Pityriasis Lichenoides; Caspase 1; Adaptor Proteins, Signal Transducing; Apoptosis Regulatory Proteins; Mycosis Fungoides; Carrier Proteins; Skin Neoplasms; Nucleotides; Interleukin-1beta; NLR Proteins
PubMed: 35776169
DOI: 10.1007/s00403-022-02363-x -
Photodermatology, Photoimmunology &... Sep 2023Pityriasis lichenoides (PL) is a papulosquamous disease affecting both children and adults, for which narrowband-UVB (NB-UVB) phototherapy is regarded as a commonly used... (Observational Study)
Observational Study
INTRODUCTION
Pityriasis lichenoides (PL) is a papulosquamous disease affecting both children and adults, for which narrowband-UVB (NB-UVB) phototherapy is regarded as a commonly used treatment option. The aim of this study was to investigate the efficacy of NB-UVB phototherapy in the management of PL and to compare response rates in pediatric and adult age groups.
MATERIALS AND METHODS
This observational, retrospective study included 20 PL patients (12 pityriasis lichenoides chronica; PLC, 8 pityriasis lichenoides et varioliformis acuta; PLEVA) who failed to respond to other treatment modalities. The data for this study were collected retrospectively from patient follow-up forms in the phototherapy unit.
RESULTS
A complete response (CR) was obtained in all pediatric patients with PL, while 53.8% of adult patients had achieved CR. The mean cumulative dose required to achieve the CR was higher in pediatric patients than adult patients with PL (p < .05). The CR was achieved in 6 (75%) of 8 PLEVA patients, while 8 (66.7%) of 12 PLC patients had reached to CR. The mean number of exposures for patients with PLC to achieve a CR was higher than patients with PLEVA (p < .05). Erythema was the most common adverse effect during phototherapy particularly in 5 (35.7%) of the patients with PL who had achieved CR.
CONCLUSIONS
NB-UVB is an effective and well-tolerated treatment option for PL especially in diffuse types. A higher response can be obtained in children with higher cumulative dose. Patients with PLC may require more exposures for CR than patients with PLEVA.
Topics: Adult; Humans; Child; Pityriasis Lichenoides; Retrospective Studies; Ultraviolet Therapy; Phototherapy; Ultraviolet Rays
PubMed: 37340660
DOI: 10.1111/phpp.12895 -
Journal of the American Academy of... Jan 2020Syphilis is often misdiagnosed clinically, and biopsies might be required.
BACKGROUND
Syphilis is often misdiagnosed clinically, and biopsies might be required.
OBJECTIVE
To determine histopathologic features that distinguish secondary syphilis from pityriasis lichenoides (PL), pityriasis rosea (PR), and early mycosis fungoides (MF).
METHODS
Histopathologic features of 100 cases of syphilis, 110 cases of PL, 72 cases of PR, and 101 cases of MF were compared.
RESULTS
Elongated rete ridges and interstitial inflammation favor syphilis over PL (likelihood ratios 3.44 and 2.72, respectively), but no feature reliably distinguishes between them. Secondary syphilis and PR can be distinguished by neutrophils in the stratum corneum, plasma cells, interface dermatitis with lymphocytes and vacuoles, and lymphocytes with ample cytoplasm. Plasma cells and lymphocytes with ample cytoplasm are rare in early MF and can be used as distinguishing features.
CONCLUSIONS
Histopathologic features characteristic of syphilis can be seen in PL, PR, and early MF. Distinguishing syphilis from PL can be difficult histologically, and a high index of suspicion is required. Although elongation of rete and interstitial inflammation favor syphilis, plasma cells (historically considered a significant feature of syphilis) are often encountered in PL. Vacuolar interface dermatitis with a lymphocyte in every vacuole is considered characteristic of PL, but this feature appears to be more common in syphilis.
Topics: Diagnosis, Differential; Humans; Mycosis Fungoides; Pityriasis Lichenoides; Pityriasis Rosea; Sensitivity and Specificity; Skin Neoplasms; Syphilis
PubMed: 31306731
DOI: 10.1016/j.jaad.2019.07.011 -
Journal of Cutaneous Pathology Apr 2024Psoriasis is an inflammatory skin disease driven by upregulation of cytokines in the Th17 pathway, including interleukin-36 (IL-36). Previous studies have highlighted...
BACKGROUND
Psoriasis is an inflammatory skin disease driven by upregulation of cytokines in the Th17 pathway, including interleukin-36 (IL-36). Previous studies have highlighted the utility of IL-36 immunostaining for psoriasis compared to spongiotic dermatitis and other psoriasiform dermatoses; however, no study has examined the role of IL-36 staining in distinguishing psoriasis from pityriasis rosea (PR) and pityriasis lichenoides (PL), known histologic mimickers of psoriasis.
METHODS
We compared the immunostaining pattern of IL-36 for 21 PR cases, 22 PL cases, and 10 psoriasis cases. We graded the immunostaining as 0, negative; 1, focal weak; 2, diffuse weak; 3, focal, strong; or 4, diffuse strong. We further categorized stains as negative (0-2 score) or positive (3-4 score) and utilized Fisher's exact test to compare the immunostaining pattern of these entities.
RESULTS
All psoriasis specimens were positive for IL-36, whereas all PR specimens were negative (p = 0.00000002). Twenty PL specimens were negative (p = 0.000001). Nine of 10 pityriasis lichenoides et varioliformis acuta cases were negative (p = 0.00012), and 11 of 12 cases of pityriasis lichenoides chronica were negative (p = 0.00003).
CONCLUSIONS
Our findings highlight the potential role of IL-36 immunostaining in distinguishing psoriasis from other psoriasiform dermatoses, including PR and PL.
PubMed: 38689501
DOI: 10.1111/cup.14633 -
Cureus Mar 2023A rare subtype of mycosis fungoides (MF) known as pityriasis lichenoides-like mycosis fungoides (PL-like MF) manifests as recurrent crops of erythematous scaly papules...
A rare subtype of mycosis fungoides (MF) known as pityriasis lichenoides-like mycosis fungoides (PL-like MF) manifests as recurrent crops of erythematous scaly papules with the histological findings of MF. We report a 64-year-old male with recurrent crops of psoriasiform papules with mild scales on his trunk and extremities. Skin biopsy results were consistent with CD8+ cutaneous T-cell lymphoma (CTCL). Our patient had clinical features of pityriasis lichenoides and histological findings consistent with CD8+ MF. A differential diagnosis of PL, lymphomatoid papulosis (LyP), and PL-like MF was considered. Counseling patients with CD8+ cutaneous T-cell lymphoma can be challenging, as there is an aggressive variant named primary cutaneous aggressive epidermotropic CD8+ CTCL. However, with the ability to recognize PL-like MF, a rare indolent type of CD8+ CTCL, physicians can counsel patients appropriately.
PubMed: 37113344
DOI: 10.7759/cureus.36665 -
Archives of Dermatological Research Nov 2019Mycosis fungoides (MF) is the most common form of cutaneous T cell lymphoma (CTCL) with many clinical variants including papular and pityriasis lichenoides chronica... (Clinical Trial)
Clinical Trial
Mycosis fungoides (MF) is the most common form of cutaneous T cell lymphoma (CTCL) with many clinical variants including papular and pityriasis lichenoides chronica (PLC)-like variants. During psoralen and ultraviolet A (PUVA) treatment of MF, PLC-like papular lesions were observed to appear. The exact nature of these lesions is not fully understood. This work aimed to study PLC-like papular lesions arising in MF patients receiving PUVA therapy clinically, histopathologically and immunohistochemically (using monoclonal antibodies against CD4 and CD8) and to compare them with lesions in classic PLC patients. Fifteen MF patients with PLC-like papular lesions arising during PUVA treatment were included and 15 patients with classic PLC served as controls. While the extent of these lesions significantly correlated with their duration (p < 0.05), it showed no significant correlation with the TNMB stage of MF, number of phototherapy sessions or cumulative UVA dose at which they started to appear. The response status of MF to PUVA did not affect their development. Compared to classic PLC, these lesions showed significantly more acute onset (p = 0.003). None of these lesions showed histopathological features essential to diagnose papular/PLC-like MF and no significant difference existed with regard to their histopathological and CD4/CD8 phenotypic features compared to classic PLC. Papular lesions mimicking PLC in MF patients receiving PUVA mostly represent an upgrading reaction with possible good prognostic implication.
Topics: Adolescent; Adult; CD4 Antigens; CD8 Antigens; Cross-Sectional Studies; Female; Humans; Immunohistochemistry; Male; Middle Aged; Mycosis Fungoides; PUVA Therapy; Pityriasis Lichenoides; Skin; Skin Neoplasms; Young Adult
PubMed: 31300833
DOI: 10.1007/s00403-019-01949-2 -
Boletin Medico Del Hospital Infantil de... 2023Pityriasis lichenoides et varioliformis acuta (PLEVA) is a rare dermatosis recognized as a benign condition of unknown etiopathogenesis. It is more common in pediatric... (Review)
Review
BACKGROUND
Pityriasis lichenoides et varioliformis acuta (PLEVA) is a rare dermatosis recognized as a benign condition of unknown etiopathogenesis. It is more common in pediatric patients and young adults and is characterized by multiple small or large erythematous plaques spread over the trunk and extremities.
CASE REPORT
We describe the case of a 5-year-old male, previously healthy, with multiple erythematous lesions that disappeared leaving hypopigmented macules. The biopsy reported histological changes suggestive of mycosis fungoides. After a second revision of lamellae in this hospital, lymphocytic vasculitis (LV) with focal epidermal necrosis consistent with acute pityriasis lichenoides (PL) was identified.
CONCLUSIONS
The existing knowledge about PLEVA lacks a consensus in specifying its classification, etiopathogenesis, diagnosis, and treatment, so this clinical condition represents a medical challenge. The diagnosis is made by clinical suspicion and confirmed by histology. The objective of this article was to report a case of PLEVA with an atypical presentation due to its histopathological findings, being the first report showing LV in children, as well as a review of the literature.
Topics: Male; Young Adult; Humans; Child; Child, Preschool; Pityriasis; Pityriasis Lichenoides; Skin Diseases
PubMed: 37155724
DOI: 10.24875/BMHIM.22000043 -
JAAD Case Reports May 2023
PubMed: 37078017
DOI: 10.1016/j.jdcr.2023.02.006