In utero development of epidermolysis bullosa acquisita

Pediatric Dermatology, EarlyView.
Source: Pediatric Dermatology - Category: Dermatology Authors: Source Type: research

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Rhys has epidermolysis bullosa, a painful, life-limiting condition that has left him unable to walk.
Source: BBC News | Health | UK Edition - Category: Consumer Health News Source Type: news
Chronic pruritus causes major morbidity in epidermolysis bullosa (EB). The substance P-neurokinin 1 receptor (SP-NK1) pathway is a promising target for treating EB-related pruritus.
Source: Journal of the American Academy of Dermatology - Category: Dermatology Authors: Source Type: research
In conclusion, our results show that surgical correction followed by skin dressing changes is an effective approach to improving mitten-hand malformations in RDEB patients. PMID: 31502914 [PubMed - as supplied by publisher]
Source: Journal of Plastic Surgery and Hand Surgery - Category: Surgery Tags: J Plast Surg Hand Surg Source Type: research
We report a case of a 39-year-old male with autosomal dominant dystrophic EB, presenting with bilateral leg swelling of one-week duration. There was no other significant past medical history. The physical examination was remarkable for scars and erosions over all body areas, with all extremities with blisters and ulcers covered, absent finger and toenails and bilateral lower extremity edema. Serum creatinine was 0.9 mg/dL, albumin 1.3 g/dL and urine protein excretion 3.7 g/24 h. Viral markers (hepatitis-B, C, and HIV), complement c3 and c4 levels and auto-immune antibody profile all remained neg...
Source: Renal Failure - Category: Urology & Nephrology Tags: Ren Fail Source Type: research
Abstract Bullous diseases are uncommon in children; however, as they have the potential to affect quality of life, occasionally have long-term side effects in the setting of scarring processes, and carry a rare risk of underlying malignancy [e.g., with paraneoplastic pemphigus (PNP)], knowledge of their clinical presentation and treatment options is essential. Given the rarity of these conditions, our current state of knowledge is largely derived from case reports and case series, with a paucity of evidence-based recommendations. In this review, we discuss the clinical presentation of and treatment options for lin...
Source: Paediatric Drugs - Category: Pediatrics Authors: Tags: Paediatr Drugs Source Type: research
A, Has C, Guenova E PMID: 31502654 [PubMed - as supplied by publisher]
Source: Acta Dermato-Venereologica - Category: Dermatology Authors: Tags: Acta Derm Venereol Source Type: research
AbstractBullous diseases are uncommon in children; however, as they have the potential to affect quality of life, occasionally have long-term side effects in the setting of scarring processes, and carry a rare risk of underlying malignancy [e.g., with paraneoplastic pemphigus (PNP)], knowledge of their clinical presentation and treatment options is essential. Given the rarity of these conditions, our current state of knowledge is largely derived from case reports and case series, with a paucity of evidence-based recommendations. In this review, we discuss the clinical presentation of and treatment options for linear immuno...
Source: Pediatric Drugs - Category: Pediatrics Source Type: research
We report our experience with the care of a parturient woman diagnosed with EBS. There is little literature on pregnancy in women with this condition. Special precautions are necessary during diagnostic and therapeutic interventions to avoid bullae formation or exacerbation of existing lesions. Frictional or shearing forces are typically more damaging than compressive forces. Multidisciplinary planning was done for our patient to ensure uneventful labor and delivery. Elective induction of labor was started at 40 weeks of gestation. She eventually underwent a cesarean delivery after failed trial of labor. We present this ...
Source: Case Reports in Womens Health - Category: OBGYN Source Type: research
CONCLUSION: The results highlight that nutritional compromise occurs early in children with RDEB and may, therefore, require interventions in the first year or two of life. This article is protected by copyright. All rights reserved. PMID: 31487386 [PubMed - as supplied by publisher]
Source: The British Journal of Dermatology - Category: Dermatology Authors: Tags: Br J Dermatol Source Type: research
PMID: 31486079 [PubMed - as supplied by publisher]
Source: The British Journal of Dermatology - Category: Dermatology Authors: Tags: Br J Dermatol Source Type: research
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