- Brunsting-Perry Type Pemphigoid Causing Secondary Cicatricial Alopecia in 2 Patients. [Journal Article]
- SASkin Appendage Disord 2018; 4(4):308-311
- Brunsting-Perry type pemphigoid (BPP) is a rare subepidermal blistering disease and a cause of secondary cicatricial alopecia. It was originally described by Brunsting and Perry in 1957 as a rare var...
Brunsting-Perry type pemphigoid (BPP) is a rare subepidermal blistering disease and a cause of secondary cicatricial alopecia. It was originally described by Brunsting and Perry in 1957 as a rare variant of cicatricial pemphigoid, characterized by bullous lesions limited to the head, neck, scalp, and upper trunk with mild or no mucosal involvement. We report 2 cases of BPP cicatricial alopecia with histopathology of subepidermal blister formation, different clinical presentation, and different salt-split test results. One patient had features of bullous pemphigoid (BP) with important oral mucosal involvement (not yet reported in the literature), and the second patient had typical features of epidermolysis bullosa acquisita (EBA). The secondary cicatricial alopecia may be due to different antigens associated with either BP or EBA. The phenomenon of epitope spreading could explain the association between 2 distinctive bullous diseases in the same patient, justifying the divergent findings of the immunofluorescence. The specific target antigen of BPP is yet to be defined.
- Topical sucralfate cream treatment for aplasia cutis congenita with dystrophic epidermolysis bullosa: a case study. [Journal Article]
- JWJ Wound Care 2018 Nov 02; 27(11):768-771
- Bart syndrome consists of aplasia cutis congenita (ACC) and dominant or recessive dystrophic epidermolysis bullosa (DEB), associated with skin fragility and nail dysplasia. ACC in DEB is thought to b...
Bart syndrome consists of aplasia cutis congenita (ACC) and dominant or recessive dystrophic epidermolysis bullosa (DEB), associated with skin fragility and nail dysplasia. ACC in DEB is thought to be caused by trauma, the most cited cause being in utero formation of bullae consequent to friction of the limbs. Epidermolysis bullosa (EB) refers to a hereditary mechanobullous disease following trauma, characterised by formation of blisters on the skin and mucous membranes. There are four categories of the disease, including epidermolysis bullosa simplex, junctional epidermolysis bullosa, dystrophic epidermolysis bullosa and Kindler syndrome. Infection, sepsis and death may occur as a consequence of generalised blistering with complication. We present the case of a newborn diagnosed with DEB and whose lesions became almost fully epithelialised after treatment with 10% topical sucralfate.
- Bullous autoimmune dermatoses. [Journal Article]
- JDJ Dtsch Dermatol Ges 2018; 16(11):1339-1358
- Pathophysiologically, bullous autoimmune dermatoses are caused by autoantibodies directed against adhesion molecules or structural proteins of the skin and mucous membranes, clinically resulting in b...
Pathophysiologically, bullous autoimmune dermatoses are caused by autoantibodies directed against adhesion molecules or structural proteins of the skin and mucous membranes, clinically resulting in blister formation. Depending on the respective target proteins of the autoimmune response and their location in the skin, a distinction is made between intraepidermal (pemphigus disorders), junctional (pemphigoid disorders), and subepidermal (epidermolysis bullosa acquisita, dermatitis herpetiformis) autoimmune blistering diseases. The most common bullous autoimmune dermatosis, bullous pemphigoid is characterized by marked clinical variability and intense pruritus. Predominantly affecting elderly individuals, there has been a significant increase in its incidence in recent years. While mucosal lesions occur in less than 30 % of bullous pemphigoid patients, the second most common bullous autoimmune dermatosis, pemphigus vulgaris, typically presents with oral erosions as the predominant and - frequently - initial symptom. Its onset is usually in the 4th to 6th decade of life. Scarring is typically found in subepidermal blistering disorders such as epidermolysis bullosa acquisita or mucous membrane pemphigoid. Diagnosis is based on clinical and histological findings as well as direct and indirect immunofluorescence and detection of circulating autoantibodies. Although a number of controlled clinical trials have been conducted in recent years, treatment of bullous autoimmune disorders is still primarily based on clinical experience. Therapeutic options include topical and systemic corticosteroids as well as adjuvant immunosuppressants. Recalcitrant cases may require treatment with immunoadsorption, intravenous immunoglobulins, or the monoclonal anti-CD20 antibody rituximab.
- Probable cutaneous loxoscelism with mild systemic symptoms: a case report from Spain. [Journal Article]
- TToxicon 2018 Nov 01
- We present a case from Valencia, Spain, of a 25-year-old woman who presented with a painful erythematous skin lesion, initially diagnosed as cellulitis. The lesion was unresponsive to antibiotic trea...
We present a case from Valencia, Spain, of a 25-year-old woman who presented with a painful erythematous skin lesion, initially diagnosed as cellulitis. The lesion was unresponsive to antibiotic treatments and progressed into a hemorrhagic blister with necrotic ulcer formation. Posterior collection of a spider from the patient´s home and expert identification of the spider as Loxosceles rufescens was achieved, establishing the diagnosis of probable cutaneous loxoscelism. Symptomatic treatment, general wound care and ultimately surgery, resulted in complete recovery with minor residual scarring. This case illustrates some of the difficulties encountered in the diagnosis and treatment of loxoscelism and adds to the increasing reports of loxoscelism in the Mediterranean Basin.
- Paritaprevir, ritonavir, ombitasvir, and dasabuvir with and without ribavirin in people with HCV genotype 1 and recent injecting drug use or receiving opioid substitution therapy. [Journal Article]
- IJInt J Drug Policy 2018 Oct 29; 62:94-103
- CONCLUSIONS: Paritaprevir/ritonavir, ombitasvir, and dasabuvir with or without ribavirin for 12 weeks is effective among people with HCV genotype 1 with recent injecting drug use and/or receiving OST.
- Adherence to sofosbuvir and velpatasvir among people with chronic HCV infection and recent injection drug use: The SIMPLIFY study. [Journal Article]
- IJInt J Drug Policy 2018 Oct 20; 62:14-23
- CONCLUSIONS: This study demonstrated high adherence to once-daily sofosbuvir/velpatasvir therapy among a population of people with recent injecting drug use. Recent stimulant injecting prior to and during DAA therapy and inconsistent dose-timing during treatment was associated with non-adherence. However, there was no impact of non-adherence on response to therapy, suggesting that adherence is not a significant barrier to successful DAA therapy in people with recent injecting drug use.
- Immunoglobulin E and bullous pemphigoid. [Journal Article]
- EJEur J Dermatol 2018 Aug 01; 28(4):440-448
- Clinical features and histological findings in bullous pemphigoid (BP) suggest a Th2-oriented inflammatory reaction, especially in the early stages of the disease. Elevated total serum IgE levels, bl...
Clinical features and histological findings in bullous pemphigoid (BP) suggest a Th2-oriented inflammatory reaction, especially in the early stages of the disease. Elevated total serum IgE levels, blood eosinophilia, and elevated serum levels of different soluble inflammatory Th2 response mediators have been described in large cohorts of patients with classic clear-cut BP manifestations. Direct immunofluorescence, indirect immunofluorescence, and anti-BP230 and anti-BP180 IgE ELISA testing show self-reactive IgE autoantibodies in a consistent number of BP patients. Both IgE autoantibodies and a Th2-oriented immune response may play a role in the initial phases of BP and atypical cases of BP, such as severe erythematous and urticarial forms of BP, as well as blister formation. Two recently reported experimental murine models employing IgE autoantibodies against BP180 have been reported and the successful treatment of bullous pemphigoid with the anti-IgE antibody, omalizumab, supports the roles played by IgE autoantibodies in BP pathogenesis.
- Mucous Membrane Pemphigoid, Bullous Pemphigoid, and Anti-programmed Death-1/ Programmed Death-Ligand 1: A Case Report of an Elderly Woman With Mucous Membrane Pemphigoid Developing After Pembrolizumab Therapy for Metastatic Melanoma and Review of the Literature. [Journal Article]
- FMFront Med (Lausanne) 2018; 5:268
- An 83-year-old patient developed erosions and a blister of the gingival mucous membrane, 6 months after discontinuation of the anti-programmed death-1 (anti PD-1) pembrolizumab therapy administered f...
An 83-year-old patient developed erosions and a blister of the gingival mucous membrane, 6 months after discontinuation of the anti-programmed death-1 (anti PD-1) pembrolizumab therapy administered for 10 months for a metastatic melanoma. A diagnosis of mild mucous membrane pemphigoid (MMP) was made. Complete remission of MMP was rapidly obtained with minimal therapy (doxycycline). MMP remained in complete remission after a 3-month follow-up since discontinuation of the doxycycline therapy and no evidence of relapse of the melanoma was observed after a 14-month follow-up since discontinuation of the pembrolizumab therapy. The widespread use of anti PD-1 and anti-programmed death-ligand-1 (PD-L1) in several malignancies reveals new adverse events. MMP describes a group of chronic, inflammatory, mucous membrane-predominant, subepithelial auto-immune blistering diseases. It is clinically distinct from bullous pemphigoid another autoimmune blistering disease but shares some immunological similarities with it. Twenty-nine cases of bullous pemphigoid associated with anti PD-1/PD-L1 have been reported in the literature and one of MMP. Here, we described the case of a MMP developed after pembrolizumab and discussed the accountability of anti PD-1/PD-L1 in our case and the previous reported bullous pemphigoid and MMP cases using the Begaud system scoring.
- Linear IgA bullous dermatosis associated with ulcerative proctitis: treatment challenge. [Journal Article]
- DODermatol Online J 2018 Jul 15; 24(7)
- Linear IgA bullous dermatosis is a rare bullous disease in children and adults that can be associated with autoimmune conditions, malignancies, infections, or medication exposure. The definitive diag...
Linear IgA bullous dermatosis is a rare bullous disease in children and adults that can be associated with autoimmune conditions, malignancies, infections, or medication exposure. The definitive diagnosis relies on the biopsy. A 58-year-old man presented to our clinic with a pruritic vesicular and bullous eruption. Histology showed the classic findings of a subepidermal blister with neutrophilic infiltrate and linear IgA deposition along the dermal-epidermal junction. Upon further evaluation, he was diagnosed with ulcerative proctitis. His therapy was complicated owing to side effects and lack of response to the standard treatment options. Dapsone, a first-line therapy, caused symptomatic methemoglobinemia whereas niacinamide with doxycycline were not effective. He required intravenous and oral steroids to reach improvement followed by transitioning to methotrexate.
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- A comparative study between suction blistering graft, mini punch graft and hair follicle transplant in treatment of patients with stable vitiligo. [Journal Article]
- JDJ Dermatolog Treat 2018 Sep 25; :1-20
- CONCLUSIONS: This study concluded that suction blistering technique is more effective and safe than mini punch technique and hair follicle technique in treatment of stable vitiligo.