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1.
Pediatr Dermatol ; 2024 Apr 29.
Artigo em Inglês | MEDLINE | ID: mdl-38684232

RESUMO

Actinic prurigo is a rare photodermatosis characterized by pruritic papulonodular lesions. Treatment is challenging, especially in children, as sun protection strategies need to be rigorously implemented and symptoms often persist throughout the year. Herein, we present a case of actinic prurigo in an 8-year-old patient with rapid and successful relief with baricitinib.

2.
Am J Med Genet A ; 194(5): e63519, 2024 May.
Artigo em Inglês | MEDLINE | ID: mdl-38214124

RESUMO

Metabolic pathways are known to generate byproducts-some of which have no clear metabolic function and some of which are toxic. Nicotinamide adenine dinucleotide phosphate hydrate (NAD(P)HX) is a toxic metabolite that is produced by stressors such as a fever, infection, or physical stress. Nicotinamide adenine dinucleotide phosphate hydrate dehydratase (NAXD) and nicotinamide adenine dinucleotide phosphate hydrate epimerase (NAXE) are part of the nicotinamide repair system that function to break down this toxic metabolite. Deficiency of NAXD and NAXE interrupts the critical intracellular repair of NAD(P)HX and allows for its accumulation. Clinically, deficiency of NAXE manifests as progressive, early onset encephalopathy with brain edema and/or leukoencephalopathy (PEBEL) 1, while deficiency of NAXD manifests as PEBEL2. In this report, we describe a case of probable PEBEL2 in a patient with a variant of unknown significance (c.362C>T, p.121L) in the NAXD gene who presented after routine immunizations with significant skin findings and in the absence of fevers.


Assuntos
Encefalopatias , Imunização , Humanos , Imunização/efeitos adversos , Leucoencefalopatias/etiologia , Racemases e Epimerases/deficiência , Racemases e Epimerases/genética , Hidroliases/deficiência , Hidroliases/genética , Encefalopatias/etiologia
3.
Exp Dermatol ; 32(5): 599-610, 2023 05.
Artigo em Inglês | MEDLINE | ID: mdl-36691705

RESUMO

Atopic dermatitis (AD) is the most common skin inflammatory disease. Dysregulation of innate and adaptive immune systems plays a major role in the pathophysiology of AD. JAKi (Janus Kinase Inhibitors) reduce the production of pro-inflammatory cytokines and represent a promising novel treatment for AD. To assess and summarize the overall efficacy and safety of topial JAKi in the treatment of AD in adults and pediatrics, a broad search was performed on Ovid Medline, Ovid Embase, Cochrane Library, Web of Sciences, Scopus, CINAHL and Google Scholar until 14 June 2022. After screening, 19 studies remained for the final review. The current systematic review was conducted according to PRISMA, and the protocol was registered in PROSPERO (ID #CRD42022303321). Topical delgocitinib, tofacitinib, ruxolitinib, cerdulatinib and ifidancitinib are effective in treating AD and significantly improve EASI, IGA, pruritus-NRS score and some other indexes in adults. Moreover, topical delgocitinib was observed to have a great efficacy in the treatment of AD in paediatrics. All topical JAKi showed minimal risk of mild-to-moderate adverse effects. Available topical JAKi are effective and safe modalities in treating AD. Nevertheless, further studies with longer duration and head-to-head comparative trials are necessary to find the best option with the least adverse effects.


Assuntos
Dermatite Atópica , Inibidores de Janus Quinases , Pediatria , Humanos , Adulto , Criança , Dermatite Atópica/induzido quimicamente , Prurido/induzido quimicamente , Fatores de Tempo , Resultado do Tratamento
4.
Pediatr Dermatol ; 40(1): 84-89, 2023 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-36373243

RESUMO

BACKGROUND/OBJECTIVES: Pediatric lichen planus (LP) is rare with variable prevalence and atypical presentations compared to adults. Data on LP are lacking for the pediatric population in the United States. We present demographics, presentations, and treatments for a pediatric LP cohort. METHODS: We reviewed 26 patients diagnosed with LP at 20 years or younger. Treatment responses were defined as no response, partial response, and complete response. RESULTS: Demographics included 54% females and median diagnosis age of 16 years (range 6-20). Most patients presented with cutaneous LP (65%), with fewer having associated oral (23%), nail (7.7%), or genital (3.8%) involvement. Some had cutaneous-only LP (38%) or strictly mucosal LP (oral-only 19% and genital-only 15%). LP lesions were pruritic (50%), painful (19%), and/or asymptomatic (35%). Complete/partial responses occurred with medium-potency topical corticosteroids in cutaneous (n = 7; 64%), oral (n = 3; 75%), and genital LP (n = 3; 100%), with high/ultra-high potency topical corticosteroids in oral LP (n = 6; 86%), and with topical calcineurin inhibitors in genital LP (n = 2; 100%). Side effects were clobetasol-related oral candidiasis and biopsy-related penile depressed scar. Most patients with available follow-up achieved remission (n = 17; 81%). CONCLUSIONS: Pediatric LP usually presents in adolescence with cutaneous involvement and is symptomatic. However, patients frequently can have oral, genital, or nail lesions or may be asymptomatic, so they need thorough examinations and follow-up. Long-term remission is common due to treatment or natural disease course. Medium-potency corticosteroids are recommended for cutaneous, oral, and genital LP. Various other local and systemic therapies exist with successful treatment responses.


Assuntos
Líquen Plano Bucal , Líquen Plano , Adulto , Feminino , Humanos , Criança , Adolescente , Adulto Jovem , Masculino , Seguimentos , Estudos Retrospectivos , Líquen Plano/diagnóstico , Líquen Plano/tratamento farmacológico , Líquen Plano/patologia , Líquen Plano Bucal/diagnóstico , Líquen Plano Bucal/tratamento farmacológico , Corticosteroides/uso terapêutico
6.
Clin Case Rep ; 10(6): e05910, 2022 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-35662780

RESUMO

Development of pemphigus foliaceus (PF) following SARS-CoV-2 infection has only been reported in one patient who had received Bamlanivimab and thus might be considered as a drug-induced case of PF. Here, we reported the first case of PF arising solely after COVID infection without taking any culprit drug.

7.
Dermatol Ther ; 35(8): e15625, 2022 08.
Artigo em Inglês | MEDLINE | ID: mdl-35674693

RESUMO

Lichen planopilaris (LPP) is a chronic inflammatory disorder of hair without a proven effective and safe treatment. To objectively assess the clinical efficacy of mycophenolate mofetil (MMF) in patients suffering from LPP, a retrospective cohort study was conducted on 52 patients who treated with MMF (2 g/day) at least for 6 months. LPP activity index (LPPAI) before and after treatment was calculated and compared. Most of the patients were female and belonged to the age group of 50-60 years. All of the disease activity indices were significantly improved after 6 months of therapy (p < 0.001). The majority of patients had LPPAI 4-6 and 0-2, before and after treatment, respectively. After 6 months of treatment, half of patients showed a disease activity decrease (LPPAI reduced >25% compared to the baseline value). Systemic MMF is an effective and relatively safe treatment modality for patients with LPP and could lead to significant reduction in disease activity regarding both subjective and objective indices.


Assuntos
Líquen Plano , Feminino , Humanos , Imunossupressores/efeitos adversos , Líquen Plano/induzido quimicamente , Líquen Plano/diagnóstico , Líquen Plano/tratamento farmacológico , Masculino , Pessoa de Meia-Idade , Ácido Micofenólico/efeitos adversos , Estudos Retrospectivos , Resultado do Tratamento
9.
Dermatol Ther ; 35(5): e15430, 2022 05.
Artigo em Inglês | MEDLINE | ID: mdl-35261123

RESUMO

SARS-CoV2 vaccines were approved without long-term monitoring due to emergent situations. This has raised some issues about timing and protocol of receiving vaccines in specific situations including patients with chronic inflammatory disorders such as psoriasis. Here, we present different aspects of SARS-CoV-2 infection and vaccination in psoriasis patients and aim to provide solutions to overcome the potential challenges. In brief, the benefits of vaccination outweigh the potential risk; vaccine-triggered de novo or flares of psoriasis is uncommon. As such, all psoriasis patients, especially those receiving systemic treatments including anti tumor necrosis factor agents, are strongly recommended to get SARS-CoV-2 vaccines. It is recommended that new immunosuppressive/immunomodulatory therapies be initiated at least 1 week after the second SARS-CoV-2 vaccine dose, if possible. In addition, in severe and active forms of psoriasis, it is better to delay vaccination until stabilization of the disease.


Assuntos
COVID-19 , Psoríase , COVID-19/prevenção & controle , Vacinas contra COVID-19/efeitos adversos , Humanos , Psoríase/tratamento farmacológico , RNA Viral/uso terapêutico , SARS-CoV-2 , Vacinação
10.
Dermatol Ther ; 35(6): e15433, 2022 06.
Artigo em Inglês | MEDLINE | ID: mdl-35266262

RESUMO

The prevalence of hair loss has increased during COVID-19. In this study, we review the current literature on incidence and characteristics of various types of COVID-19-related and COVID-19-vaccine- related hair loss including telogen effluvium, alopecia areata, friction alopecia and anagen effluvium. Regarding most of them, the more severe the infection, the more profound and prolonged the course of alopecia. However, the most important issue is reassuring the patients of the non-serious nature of this complication, since psychological support is the most important factor in the earlier resolution of the condition.


Assuntos
Alopecia em Áreas , COVID-19 , Vacinas , Alopecia/complicações , Alopecia em Áreas/epidemiologia , COVID-19/prevenção & controle , Humanos , SARS-CoV-2
11.
Indian J Dermatol ; 59(4): 334-8, 2014 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-25071248

RESUMO

BACKGROUND: Papillomatosis is a known histopathologic pattern usually seen in human papillomavirus (HPV) infection and verruca vulgaris is the typical example. This pattern is also detected in some other benign cutaneous lesions such as nevus sebaceous (NS), seborrheic keratosis (SK), trichilemmoma (TL) and inverted follicular keratosis (IFK). The association between papillomatous lesions and HPV infection is questionable. OBJECTIVE: The objective of this study was to investigate the presence of HPV deoxyribonucleic acid (DNA) in non-genital benign papillomatous skin lesions (NS, SK, TL and IFK) by polymerase chain reaction (PCR). MATERIALS AND METHODS: A total of 100 specimens of non-genital NS, SK, TL and IFK were retrieved from archives of Dermatopathology Department of Razi Hospital, between 2003 and 2010. The conventional PCR using consensus GP5+/GP6+ primer and hydroxymethylbilane synthase gene as inner control was performed. RESULTS: PCR for HPV DNA revealed no positive results in any of 28 seborrheic keratosis (SK), 28 nevus sebaceous (NS), 28 inverted follicular keratosis (IFK) and 13 trichilemmoma (TL) studied specimens. CONCLUSION: Papillomatosis is usually a characteristic pattern of HPV infection. However, we found no association between HPV infection and non-genital benign papillomatous lesions.

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