Mapeo por inmunofluorescencia para el diagnóstico de epidermólisis ampollosa congénita

Cepeda‐Valdés, Rodrigo; Pohla‐Gubo, G.; Borbolla-Escoboza, José R.; Barboza-Quintana, Oralia; Áncer‐Rodríguez, Jesús; Hintner, Helmut; Salas‐Alanís, Julio C.

doi:10.1016/j.ad.2010.06.003

Cited by 8 publications

(3 citation statements)

References 21 publications

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“…Immunofluorescent staining of the skin is utilized to determine the zone of dermoepidermal junction and to establish presence, reduction, or absence of structural skin proteins. 6 While the clinical picture is highly variable, diagnosis relies on direct IF microscopy of a perilesional skin biopsy. Histopathology by microscopy of a lesional skin biopsy allows distinguishing between intraepidermal and subepidermal blistering characteristic for pemphigoid disorders such as EB.…”

Section: Discussionmentioning

confidence: 99%

A case of a patient with severe epidermolysis bullosa surviving to adulthood

Hubail¹,

Belkharoeva²,

Tepluk³

et al. 2018

IJGM

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PurposeThe aim of this study was to evaluate the progression of a case of a patient with epidermolysis bullosa (EB) since early age who survived to adulthood, presenting with recurrent skin blisters and disfiguring scars and disabling musculoskeletal deformities.BackgroundEB is a rare group of inherited diseases that affect the skin fragility causing it to blister in response to even minor trauma. Established novel treatments are limited in the literature due to its rarity, and more research is needed to set a global management approach. Clinical manifestations range widely from localized to generalized blistering.MethodsA rare case of EB surviving to adulthood despite the complications, which has been evaluated, treated during a relapse, and followed up.ConclusionThe described case is of considerable clinical interest due to its rarity and severity. Optimal management requires a multidisciplinary approach and revolves around the protection of the skin against slightest injury, use of careful wound care dressings, aggressive nutritional support, and early medical or surgical interventions if needed to manage any complications. Prognosis varies considerably depending on each case.

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Section: Discussionmentioning

confidence: 99%

A case of a patient with severe epidermolysis bullosa surviving to adulthood

Hubail¹,

Belkharoeva²,

Tepluk³

et al. 2018

IJGM

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“…Current clinical practice focusses on the control of symptoms, signs, and complication through topical treatment. Wound care, fragile skin protection, pain and itch management, dryness, infection control, and trauma prevention are crucial [ 13 , 14 , 15 ].…”

Section: Introductionmentioning

confidence: 99%

“…In fact, the current local therapeutic is controversial and complex, mainly focused on a bandage application to protect the lesions and symptoms controls (especially pain and itching) and the application of diacerein [ 20 ], Oleogel S-10 [ 21 ], allantoin (e.g., product SD-101) [ 22 ], calcipotriol [ 23 ], Beremagene geperpavec (B-VEC) [ 24 ], silicone dressings or hydrogel dressings or with dialkylcarbamoyl chloride (DACC) [ 6 , 25 ] or henna [ 26 ], among others [ 27 , 28 , 29 ]. Considering the above, this work is considered relevant because the topical treatments used are experimental, as there is no consensus on their effectiveness, which allows the establishment of a standardised action protocol [ 5 , 15 ].…”

Section: Introductionmentioning

confidence: 99%

Management of Skin Lesions in Patients with Epidermolysis Bullosa by Topical Treatment: Systematic Review and Meta-Analysis

Pabón-Carrasco,

Caceres-Matos,

Roche-Campos

et al. 2024

Healthcare

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Epidermolysis bullosa (EB) is the overarching term for a set of rare inherited skin fragility disorders that result from mutations in at least 20 different genes. Currently, there is no cure for any of the EB subtypes associated with various mutations. Existing therapies primarily focus on alleviating pain and promoting early wound healing to prevent potential complications. Consequently, there is an urgent need for innovative therapeutic approaches. The objective of this research was to assess the efficacy of various topical treatments in patients with EB with the goal of achieving wound healing. A secondary objective was to analyse the efficacy of topical treatments for symptom reduction. A literature search was conducted using scientific databases, including The Cochrane Library, Medline (Pubmed), Web of Science, CINHAL, Embase, and Scopus. The protocol review was registered in PROSPERO (ID: 418790), and inclusion and exclusion criteria were applied, resulting in the selection of 23 articles. Enhanced healing times were observed compared with the control group. No conclusive data have been observed on pain management, infection, pruritus episodes, and cure rates over time. Additionally, evidence indicates significant progress in gene therapies (B-VEC), as well as cell and protein therapies. The dressing group, Oleogel S-10, allantoin and diacerein 1%, were the most represented, followed by fibroblast utilisation. In addition, emerging treatments that improve the patient’s innate immunity, such as calcipotriol, are gaining attention. However, more trials are needed to reduce the prevalence of blistering and improve the quality of life of individuals with epidermolysis bullosa.

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