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Pediatric Dermatology 2024Pityriasis lichenoides (PL) is an uncommon skin rash. PL has two main forms: Pityriasis lichenoides et varioliformis acuta (PLEVA): this "acute" (fast) form comes on...
Pityriasis lichenoides (PL) is an uncommon skin rash. PL has two main forms: Pityriasis lichenoides et varioliformis acuta (PLEVA): this "acute" (fast) form comes on quickly. Pityriasis lichenoides chronica (PLC): this "chronic" (long) form often develops slowly and lasts longer.
Topics: Humans; Pityriasis Lichenoides; Child; Diagnosis, Differential
PubMed: 38743416
DOI: 10.1111/pde.15645 -
Clinical Reviews in Allergy & Immunology Dec 2023Recent advances in medical genetics elucidated the background of diseases characterized by superficial dermal and epidermal inflammation with resultant aberrant... (Review)
Review
Recent advances in medical genetics elucidated the background of diseases characterized by superficial dermal and epidermal inflammation with resultant aberrant keratosis. This led to introducing the term autoinflammatory keratinization diseases encompassing entities in which monogenic mutations cause spontaneous activation of the innate immunity and subsequent disruption of the keratinization process. Originally, autoinflammatory keratinization diseases were attributed to pathogenic variants of CARD14 (generalized pustular psoriasis with concomitant psoriasis vulgaris, palmoplantar pustulosis, type V pityriasis rubra pilaris), IL36RN (generalized pustular psoriasis without concomitant psoriasis vulgaris, impetigo herpetiformis, acrodermatitis continua of Hallopeau), NLRP1 (familial forms of keratosis lichenoides chronica), and genes of the mevalonate pathway, i.e., MVK, PMVK, MVD, and FDPS (porokeratosis). Since then, endotypes underlying novel entities matching the concept of autoinflammatory keratinization diseases have been discovered (mutations of JAK1, POMP, and EGFR). This review describes the concept and pathophysiology of autoinflammatory keratinization diseases and outlines the characteristic clinical features of the associated entities. Furthermore, a novel term for NLRP1-associated autoinflammatory disease with epithelial dyskeratosis (NADED) describing the spectrum of autoinflammatory keratinization diseases secondary to NLRP1 mutations is proposed.
Topics: Humans; Psoriasis; Inflammation; Mutation; Immunity, Innate; Keratosis; Guanylate Cyclase; Membrane Proteins; CARD Signaling Adaptor Proteins; Interleukins
PubMed: 38103162
DOI: 10.1007/s12016-023-08971-3 -
Jornal de Pediatria Apr 2024This study aims to evaluate the characteristics and treatment response of patients with pityriasis lichenoides seen in the last 43 years in a pediatric dermatology...
OBJECTIVES
This study aims to evaluate the characteristics and treatment response of patients with pityriasis lichenoides seen in the last 43 years in a pediatric dermatology service.
METHODS
This was a retrospective, analytical, longitudinal study of patients under 15 years of age. The medical records were reviewed and data were presented as frequencies, means and variances. Student's t-test, Mann-Whitney test, Fisher's exact test, Pearson/Yates chi-square test and multivariate logistic regression model were used, with p < 0.05 considered.
RESULTS
41 patients were included, 32 (78.0%) with pityriasis lichenoides chronica (PLC), five (12.2%) with pityriasis lichenoides et varioliformis acuta (PLEVA) and four (9.8%) with clinical PLC without biopsy. The age range of school children and adolescents was 19 (46.3%) and 13 (31.7%) respectively and 27 (65.8%) were male. Two peaks of the highest frequency were observed between 2004 and 2006 (10 patients - 24.4%) and another between 2019 and 2021 (6 patients - 14.7%). There was remission in 71.9% (n = 23), with 56.6% (n = 17) of those who used antibiotic therapy and 80% (n = 4) of those who had phototherapy. The chance of remission was 13 times greater in patients with disease onset after 5 years of age.
CONCLUSIONS
The clinical form most commonly found was PLC mainly in school children and adolescents. The frequency peaks coincided with infectious outbreaks. The remission rate was satisfactory with antibiotic therapy, but higher with phototherapy. Remission was greater in patients with disease onset after 5 years of age.
PubMed: 38677323
DOI: 10.1016/j.jped.2024.03.011 -
Dermatology Reports Mar 2024In the wake of a global COVID-19 pandemic, where innovations in vaccination technology and the speed of development and distribution have been unprecedented, a wide...
In the wake of a global COVID-19 pandemic, where innovations in vaccination technology and the speed of development and distribution have been unprecedented, a wide variety of post-vaccination cutaneous reactions have surfaced. However, there has not been a systematic review that investigates pityriasis eruptions and the associated variants following COVID-19 inoculations. A PubMed search using was performed to find case reports from the earliest record through November 2022. Data including types of vaccination and pityriasis were extracted and a quality review was performed; 47 reports with 94 patients were found: 64.9% had pityriasis rosea (PR), 3.2% PR-like eruptions, 16.0% pityriasis rubra pilaris, 7.4% pityriasis lichenoides et varioliformis acuta, 3.2% pityriasis lichenoides chronica, and 5.3% had reactions described as . The top three COVID-19 vaccinations reported were Pfizer-BioNTech (47.9%), Oxford-AstraZeneca (11.7%), and Moderna (8.5%). Pityriasis reactivity was reported most frequently after the Pfizer-BioNTech vaccination, with pityriasis rosea being the most common variant. A large difference was additionally found between the ratio of post-vaccination pityriasis reactions following Pfizer and Moderna vaccinations (5.63), and the ratio of Pfizer's usage in the United States as of December 28, 2022 relative to that of Moderna (1.59). Further studies with adequate follow-up periods and diagnostic testing will thus need to be performed to elucidate the root of this discrepancy and better characterize the association between different pityriasis reactions and COVID-19 vaccinations.
PubMed: 38623364
DOI: 10.4081/dr.2023.9742 -
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 -
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 -
Frontiers in Medicine 2023Since the early 1990s, Ultraviolet (UV) A1 phototherapy has been described as an effective and safe treatment of a multitude of skin disorders. However, after...
The realistic positioning of UVA1 phototherapy after 25 years of clinical experience and the availability of new biologics and small molecules: a retrospective clinical study.
BACKGROUND
Since the early 1990s, Ultraviolet (UV) A1 phototherapy has been described as an effective and safe treatment of a multitude of skin disorders. However, after 30 years, its use has remained limited to few dermatological centers.
OBJECTIVE
To analyze the changes over the years and the current position of UVA1 phototherapy through a Real-World Evidence (RWE) study at a single tertiary referral center.
METHODS
We reviewed the medical files of 740 patients treated between 1998 and 2022. Treatment results were collected, efficacy was assessed by a grading scale and acute adverse effects were registered.
RESULTS
We treated patients with 26 different diseases. We registered marked improvement (MI) or complete remission (CR) in 42.8% of patients with morphea, 50% with Urticaria Pigmentosa, 40.7% with Granuloma annulare and 85.7% with skin sarcoidosis. Good results were obtained also in the treatment of chronic Graft Versus Host Disease (GVHD), Eosinophilic Fasciitis, Sclero-atrophic Lichen, skin manifestations of systemic lupus erythematosus and psoriasis of HIV+ patients. Systemic Sclerosis, Romberg's Syndrome, Bushke's Scleredema, Nephrogenic Fibrosing Dermopathy, REM Syndrome, Follicular Mucinosis, Pretibial Myxedema, Scleromyxedema, pemphigus foliaceus, chronic cutaneous lupus erythematosus, erythroderma of Netherton Syndrome and Necrobiosis Lipoidica were no or poorly responsive. In clinical indications where UVA1 was used as a second line phototherapy after narrow-band (NB)-UVB, we saw good MI or CR rates in Mycosis Fungoides (57% of patients), Atopic Dermatitis (33.9%), Pitiryasis Lichenoides chronica (50%), Pityriasis Lichenoides et varioliformis acute (75%) and Lymphomatod Papulosis (62.5%). Short-term adverse events were uncommon and mild.
CONCLUSION
Over the past decade, the annual number of treated patients has progressively declined for several reasons. Firstly, UVA1 phototherapy has taken a backseat to the cheaper and more practical NB-UVB phototherapy, which has proven effective for common indications. Secondly, the emergence of new, safe, and effective drugs for conditions such as atopic dermatitis, GVHD, and connective tissue disorders. Finally, our research has shown that UVA1 therapy is often ineffective or minimally effective for some rare diseases, contrary to previous case reports and small case series. Nonetheless, UVA1 continues to be a valuable treatment option for patients with specific skin disorders.
PubMed: 38076241
DOI: 10.3389/fmed.2023.1295145 -
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