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    Neutrophilic dermatoses: a broad spectrum of disease.
    G Ital Dermatol Venereol 2018 Jan 24. Epub 2018 Jan 24.
    Dermatology Unit Ospedale Carlo Urbai Jesi, AREA Vasta 2 ASUR Marche, Jesi, Ancona, Italy -
    The neutrophilic dermatoses (NDs) comprise a group of heterogeneous disorders characterized by inflammatory skin lesions that histologically show an intense inflammatory infiltrate composed primarily by neutrophils, with no evidence of infection or vasculitis. Although there are distinct clinical differences in the classical lesions of these disorders, many patients have overlapping features. In this review, we describe the clinical aspects of the main NDs, including: Sweet's syndrome, neutrophilic dermatosis of the dorsal hands, pyoderma gangrenosum, erythema elevatum diutinum, subcorneal pustular dermatosis, neutrophilic eccrine hidradenitis, rheumatoid neutrophilic dermatitis, neutrophilic panniculitis, and aseptic abscesses including their association with underlying diseases and the differential diagnoses. Read More

    Lichen planus pemphigoides treated with ustekinumab.
    Cutis 2017 Dec;100(6):415-418
    Advanced Desert Dermatology/Midwestern University, Glendale, Arizona, USA.
    A 71-year-old woman presented with pink to violaceous, flat-topped, polygonal papules on the volar wrists, extensor elbows, and bilateral lower legs of 3 years' duration. She also had erythematous, violaceous, infiltrated plaques with microvesiculation on the bilateral thighs of several months' duration. She reported pruritus, burning, and discomfort. Read More

    Downregulation of interferon-γ-induced protein 10 in the tears of patients with Stevens-Johnson syndrome with severe ocular complications in the chronic stage.
    BMJ Open Ophthalmol 2017 7;1(1):e000073. Epub 2017 Aug 7.
    Departments of Frontier Medical Science and Technology for Ophthalmology, Kyoto Prefectural University of Medicine, Kyoto, Japan.
    Objectives: Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are acute inflammatory vesiculobullous reactions of the skin and mucosa such as the ocular surface, oral cavity and genitals. Severe ocular complications (SOC) arise in some patients with SJS/TEN diagnosed by dermatologists. To investigate the pathophysiology of ocular surface inflammation in SJS/TEN with SOC in the chronic stage, we examined cytokines in the tears of patients with ocular surface diseases and healthy controls. Read More

    Bullous lung diseases as a risk factor for lung cancer: A case report.
    Vojnosanit Pregl 2016 Dec;73(12):1160-3
    Introduction: A possible association between lung cancer and bullous lung disease has been suggested and recently supported by the results of genetic studies.

    Case Report: A previously healthy 43-year-old man, smoker, was diagnosed with bullous lung disease at the age of 31 years. He was followed up for 12 years when lung cancer (adenocarcinoma) was found at the site. Read More

    The use of suction blisters to measure sunscreen protection against UVR-induced DNA damage.
    J Photochem Photobiol B 2018 Feb 22;179:1-6. Epub 2017 Dec 22.
    Centre de Recherche sur la Peau, Pierre Fabre Dermo-Cosmétique, F-31000 Toulouse, France.
    The formation of DNA photoproducts caused by solar UVR exposure needs to be investigated in-vivo and in particular in order to assess sunscreens' level of protection against solar genotoxicity. The study's purposes were: i) to evaluate if the roof of suction blisters is an appropriate sampling method for measuring photoproducts, and ii) to measure in-vivo sunscreen protection against cyclobutane pyrimidine dimers. Skin areas on the interior forearms of eight healthy volunteers were exposed in-vivo to 2 MED of simulated solar radiation (SSR) and to 15 MED on a sunscreen protected area. Read More

    Neutrophilic dermatoses: Kids are not just little people.
    Clin Dermatol 2017 Nov - Dec;35(6):541-554. Epub 2017 Aug 3.
    Department of Dermatology, University of Connecticut Health Center, Farmington, CT. Electronic address:
    Neutrophilic dermatoses are a group of inflammatory skin disorders characterized by an overactive innate immune system with dysregulation of neutrophils without underlying infectious etiology. The major representative conditions discussed are Sweet syndrome; pyoderma gangrenosum; neutrophilic eccrine hidradenitis; palmoplantar eccrine hidradenitis; subcorneal pustular dermatoses; bowel-associated dermatosis arthritis syndrome; and synovitis, acne, pustulosis, hyperostosis, and osteitis. We will also discuss other neutrophilic conditions present almost exclusively in the pediatric population, including congenital erosive and vesicular dermatosis with reticulated supple scarring and the recently described group of autoinflammatory diseases. Read More

    A Novel Mutation in Junctional Plakoglobin Causing Lethal Congenital Epidermolysis Bullosa.
    J Pediatr 2017 Dec;191:266-269.e1
    Department of Pediatrics, Columbia University, New York, NY; Department of Pathology and Cell Biology, Columbia University, New York, NY.
    We report a case of neonatal generalized erythema and epidermolysis resulting from a novel mutation in the junctional plakoglobin gene causing truncation of the plakoglobin protein. Expedited genetic testing enabled diagnosis while the patient was in the neonatal intensive care unit, providing valuable information for the clinicians and family. Read More

    Aggressive periodontitis associated with Kindler syndrome in a large Kindler syndrome pedigree.
    Turk J Pediatr 2017 ;59(1):56-61
    Departments of Dermatology, Faculty of Medicine, Dicle University, Diyarbakır, Turkey.
    Talo-Yıldırım T, Acun-Kaya F, Taşkesen M, Dündar S, Bozoğlan A, Tekin GG, Akdeniz S. Aggressive periodontitis associated with Kindler syndrome in a large Kindler syndrome pedigree. Turk J Pediatr 2017; 59: 56-61. Read More

    Dyshidrosiform pemphigoid localized on the hands in a child: a rare occurrence.
    An Bras Dermatol 2017 Sep-Oct;92(5):714-716
    Pt. B. D. Sharma, Post Graduate Institute of Medical Sciences - Rohtak- Haryana, India.
    Dyshidrosiform pemphigoid is an acquired autoimmune variant of bullous pemphigoid with persistent vesicobullous eruptions localized on the palms or soles, or both. It generally occurs in the elderly and is rarely reported in childhood. Hereby, we describe the first case of dyshidrosiform pemphigoid in a 12-year-old child, which was limited to the dorsal hands and treated successfully with dapsone (diaminodiphenyl sulfone). Read More

    Stevens-Johnson syndrome and toxic epidermal necrolysis: epidemiological and clinical outcomes analysis in public hospitals.
    An Bras Dermatol 2017 Sep-Oct;92(5):661-667
    Stricto Sensu Postgraduate Program In Health Sciences of Fundação de Ensino e Pesquisa em Ciências da Saúde (FEPECS) - Brasília (DF), Brazil.
    Background: Adverse drug reactions are harmful and involuntary responses to drugs that occur at doses normally used for a given condition. Among them are Stevens-Johnson syndrome and toxic epidermal necrolysis, both rare and potentially fatal conditions.

    Objectives: To analyze the epidemiological and clinical characteristics related to patients diagnosed with Stevens-Johnson syndrome and toxic epidermal necrolysis in public hospitals in the Federal District - Brazil. Read More

    [A case of Orf disease complicated by erythema multiforme].
    Arch Pediatr 2017 Dec 16;24(12):1241-1243. Epub 2017 Nov 16.
    Service d'accueil des urgences, American Memorial Hospital, CHU de Reims, 47, rue Cognacq-Jay, 51100 Reims, France.
    Ecthyma contagiosum, or Orf, is a specific infection of small ruminants accidentally reaching humans. Few cases have been reported in the literature to date. We report here a case of ecthyma contagiosum occurred in a 9-year-old girl after contact with a flock of ecthyma sheep. Read More

    Evaluation of anti-desmoglein-1 and anti-desmoglein-3 autoantibody titers in pemphigus patients at the time of the initial diagnosis and after clinical remission.
    Medicine (Baltimore) 2017 Nov;96(46):e8801
    Unit of Dermatology, University of Padua, Padova, Italy.
    It has been suggested that anti-desmoglein autoantibody titers could be helpful in follow-up and therapeutic management of pemphigus patients. However, there is no consensus regarding the relationship between anti-desmoglein autoantibody titers and clinical activity of pemphigus.The aim of our study was to evaluate if clinical remission of pemphigus relates to the presence of anti-desmoglein autoantibodies. Read More

    Association between pemphigus and psoriasis: A population-based large-scale study.
    J Am Acad Dermatol 2017 Dec;77(6):1174-1175
    Department of Quality Measurements and Research, Chief Physician's Office, Clalit Health Services, Tel Aviv, Israel; Siaal Research Center for Family Medicine and Primary Care, Faculty of Health Sciences, Ben-Gurion University of the Negev, Beer-Sheva, Israel.

    Diagnostic Utility and Pitfalls of Tzanck Smear Cytology in Diagnosis of Various Cutaneous Lesions.
    J Cytol 2017 Oct-Dec;34(4):179-182
    Department of Pathology and Laboratory Medicine, All India Institute of Medical Sciences, Bhopal, Madhya Pradesh, India.
    Aims: Tzanck smear is an old but useful test for diagnosis of cutaneous dermatoses. The aim of this study was to highlight the potential usefulness and diagnostic pitfalls of Tzanck smear for diagnosis of cutaneous dermatoses and infections.

    Materials And Methods: This hospital based cross-sectional study was carried out on all Tzanck smears received for a period of twenty months (January 2014-August 2015). Read More

    Successful and well-tolerated bi-weekly immunoadsorption regimen in pemphigus vulgaris.
    Atheroscler Suppl 2017 Nov 3;30:271-277. Epub 2017 Jun 3.
    Department of Dermatology, University Hospital and Carl Gustav Carus Faculty of Medicine, Technische Universität Dresden, Dresden, Germany. Electronic address:
    Background: Pemphigus vulgaris is a chronic autoimmune disease characterized by blisters and erosions forming in the mucous membranes and the skin. Many patients are severely impaired by pain, weight loss and increased risk of infections. The disease is mediated by specific autoantibodies directed against desmogleins that contribute to connect keratinocytes in the epidermis. Read More

    Desmoglein 3-Reactive B Cells "Hiding" in Pemphigus Lesions.
    J Invest Dermatol 2017 Nov;137(11):2255-2257
    Department of Dermatology, Keio University School of Medicine, Shinjuku-ku, Tokyo, Japan. Electronic address:
    Pemphigus vulgaris is an autoimmune blistering disease caused by anti-desmoglein 3 IgG autoantibodies. It is accepted that interactions between autoreactive B and T cells are key to humoral autoimmunity targeting desmoglein 3. This orchestrated process usually occurs in secondary lymphoid organs, including the spleen and lymph nodes. Read More

    Spectrum of orocutaneous disease associations: Immune-mediated conditions.
    J Am Acad Dermatol 2017 Nov;77(5):795-806
    Division of Dermatology, Baylor University Medical Center, Dallas, Texas. Electronic address:
    There are a number of diseases that manifest both on the skin and the oral mucosa, and therefore the importance for dermatologists in clinical practice to be aware of these associations is paramount. In the following continuing medical education series, we outline orocutaneous disease associations with both immunologic and inflammatory etiologies. Read More

    The risk of Stevens-Johnson syndrome and toxic epidermal necrolysis in new users of antiepileptic drugs.
    Epilepsia 2017 12 13;58(12):2178-2185. Epub 2017 Oct 13.
    Basel Pharmacoepidemiology Unit, Division of Clinical Pharmacy and Epidemiology, Department of Pharmaceutical Sciences, University of Basel, Basel, Switzerland.
    Objective: Older antiepileptic drugs (AEDs) are known to cause Stevens-Johnson syndrome and toxic epidermal necrolysis (SJS/TEN). However, evidence for newer AED is sparse. We quantified risks of SJS/TEN in association with use of all AEDs in the United Kingdom. Read More

    Blistering rash in a young male child.
    S Afr Med J 2017 Jun 30;107(7):578-580. Epub 2017 Jun 30.
    Paediatrics Department, Leratong Hospital, Johannesburg, South Africa.
    A 3-year-old child was brought to the casualty department at Leratong Hospital, Johannesburg, South Africa, with severe bullous eruptions on the distal portions of both the upper and lower limbs. He had visited three different general practitioners prior to presentation and had been given several drugs and ointments, including three different classes of antibiotics. During his admission, the diagnosis of linear IgA bullous dermatitis was made. Read More

    First report of clinical presentation of a bite by a running spider, Philodromus sp. (Araneae: Philodromidae), with recommendations for spider bite management.
    S Afr Med J 2017 Jun 30;107(7):576-577. Epub 2017 Jun 30.
    Wits Research Institute for Malaria, School of Pathology, Faculty of Health Sciences, University of the Witwatersrand, Johannesburg, South Africa; Centre for Emerging, Zoonotic and Parasitic Diseases, National Institute for Communicable Diseases, Johannesburg, South Africa.
    This article describes the clinical progression of symptoms over a period of 5 days of a bite inflicted by a Philodromus sp. spider. Commonly known as 'running spiders', these are not considered to be harmful to humans. Read More

    A comparative study of expression of Fc receptors in relation to the autoantibody-mediated immune response and neutrophil elastase expression in autoimmune blistering dermatoses.
    Pol J Pathol 2017;68(2):109-116
    Here we investigated the cutaneous CD32A and CD89 expression in relation to the neutrophil elastase (NE) expression and serum level of anti-desmoglein 1 and 3 (DSG1/DSG3) IgG in pemphigus, anti-BP180/BP230 IgG in bullous pemphigoid (BP), anti-gliadin nonapeptides (npG), tissue (tTG), and epidermal transglutaminases (eTG) IgA in dermatitis herpetiformis (DH). The examined material consisted of skin/mucosal tissues and sera. In total, 87 patients were studied. Read More

    Spatial Distribution of Pemphigus Occurrence over Five Decades in Southeastern Brazil.
    Am J Trop Med Hyg 2017 Dec 21;97(6):1737-1745. Epub 2017 Sep 21.
    Department of Maternal-Infant Nursing and Public Health, University of São Paulo, Ribeirão Preto, São Paulo, Brazil.
    Well-defined locations of pemphigus cases support the hypothesis of environmental factors' involvement in its etiopathogenesis; however, these foci have never been described using specialized geographical tools. This is the first report to geo-reference pemphigus cases in a high-prevalence Brazilian region using geographic information systems. We aimed to report the spatio-temporal behavior of pemphigus foliaceus (PF) and vulgaris (PV) in southeastern Brazil, over the last five decades to describe geographical clusters, as well as to characterize the land use in the city with the highest number of cases. Read More

    Case Report: Bullous Scabies in Two Children below 10 Years.
    Am J Trop Med Hyg 2017 Dec 21;97(6):1746-1748. Epub 2017 Sep 21.
    Department of Dermatology, The Eastern Hospital of The First Affiliated Hospital, Sun Yat-sen University, Guangzhou, China.
    Bullous scabies is an infrequent and atypical presentation of scabies, with predilection for elderly and males. Its median age of presentation is 70 years. We report two male cases of bullous scabies who were 7 years and 6 months old. Read More

    Two-year Results After Deep Sclerectomy With Nonabsorbable Uveoscleral Implant (Esnoper-Clip): Surgical Area Analysis Using Anterior Segment Optical Coherence Tomography.
    J Glaucoma 2017 Oct;26(10):929-935
    Ophthalmology department, carretera de Canyet, S/N, 08916 Badalona, Barcelona, Spain.
    Purpose: To report 2-year results of deep sclerectomy (DS) with mitomycin C and the uveoscleral implant Esnoper-Clip (AJL Ophthalmics, Álava, Spain), a nonabsorbable hema implant, and the morphologic analysis of the surgical area with anterior segment optical coherence tomography (AS-OCT).

    Materials And Methods: In total, 41 eyes of 41 patients with medically uncontrolled open angle glaucoma who underwent DS with the uveoscleral implant were included in this prospective interventional study. Patients were recruited and selected sequentially. Read More

    Young donor-graft assisted endothelial keratoplasty (PDEK/DMEK) with epithelial debridement for chronic pseudophakic bullous keratopathy.
    Can J Ophthalmol 2017 Oct 16;52(5):519-526. Epub 2017 May 16.
    Dr. Agarwal's Eye Hospital & Research Centre, Chennai, India.
    Objective: The aim of this study was to describe the applicability and report visual outcomes for the treatment of subepithelial fibrosis and anterior stromal scarring in cases of chronic pseudophakic bullous keratopathy (PBK) with epithelial debridement and endothelial keratoplasty (EK) (pre-Descemet endothelial keratoplasty [PDEK]; Descemet membrane endothelial keratoplasty [(DMEK]) using young donor tissue.

    Design: Prospective, single-centre, interventional study.

    Participants: 6 cases with chronic PBK (> 1 year duration). Read More

    Recurrent impetigo herpetiformis: case report.
    Pan Afr Med J 2017 24;27:219. Epub 2017 Jul 24.
    Faculty of Health Sciences, Egerton University, Njoro, Kenya.
    Impetigo herpetiformis (pustular psoriasis of pregnancy) is a rare dermatosis of pregnancy that typically starts in the 2half of pregnancy and resolves postpartum. It may recur in subsequent pregnancies. I present a case of 23 year old female gravida 4 para 3 with recurrent impetigo herpetiformis at 26 weeks gestation. Read More

    An RNA-targeted therapy for dystrophic epidermolysis bullosa.
    Nucleic Acids Res 2017 Sep;45(17):10259-10269
    EB House Austria, Research Program for Molecular Therapy of Genodermatoses, Department of Dermatology, University Hospital of the Paracelsus Medical University Salzburg, Austria.
    Functional impairment or complete loss of type VII collagen, caused by mutations within COL7A1, lead to the severe recessive form of the skin blistering disease dystrophic epidermolysis bullosa (RDEB). Here, we successfully demonstrate RNA trans-splicing as an auspicious repair option for mutations located in a wide range of exons by fully converting an RDEB phenotype in an ex vivo pre-clinical mouse model based on xenotransplantation. Via a self-inactivating (SIN) lentiviral vector a 3' RNA trans-splicing molecule, capable of replacing COL7A1 exons 65-118, was delivered into type VII collagen deficient patient keratinocytes, carrying a homozygous mutation in exon 80 (c. Read More

    [Epidermolysis bullosa: oral manifestations and their treatments].
    Orv Hetil 2017 Oct;158(40):1577-1583
    Konzerváló és Esztétikai Fogászati Tanszék, Szegedi Tudományegyetem, Fogorvostudományi Kar, Szeged, Tisza L. krt. 64-66., 6720.
    The aim of this comprehensive article is to provide guidelines for the daily treatment of patients with epidermolysis bullosa, thus contributing to the attainment of their higher quality of life through the improvement of their oral health. Moreover, it is our intention to facilitate the cooperation among Hungarian general practitioners, dermatologists and dentists. Relying on recent research findings of the international literature, we intend to help general practitioners or dermatologists treating epidermolysis bullosa patients on a daily basis by identifying symptoms that require consulting an oral professional on the one hand, and to present the most important prevention strategies and further treatments advised for dentists on the other. Read More

    Clinical evaluation of a multiparametric ELISA as a rapid tool for routinely diagnosing IgG-mediated autoimmune blistering dermatoses in ethnic Slavs.
    J Clin Lab Anal 2017 Sep 26. Epub 2017 Sep 26.
    Autoimmune Blistering Dermatoses Section, Department of Dermatology, Poznan University of Medical Sciences, Poznan, Poland.
    Background: Technical innovation of autoimmune blistering dermatoses (ABDs) diagnosis aimed at multiplex approach. Two multiparametric ELISA tests are commercially available for ABDs serology. The aim was to compare diagnostic accuracy of multiparametric and monospecific ELISAs and to examine the diagnostic value/agreement of multivariant ELISA in compliance with traditional diagnostic setup for ABDs. Read More

    Insights into the epidemiological link between biting flies and pemphigus foliaceus in southeastern Brazil.
    Acta Trop 2017 Dec 21;176:455-462. Epub 2017 Sep 21.
    Division of Dermatology, Department of Clinical Medicine, Ribeirão Preto Medical School, University of São Paulo, Ribeirão Preto, São Paulo, Brazil. Electronic address:
    Background: Black fly and sandfly bites are related to the endemicity of pemphigus foliaceus (PF); however, an immune reaction against the salivary proteins from these flies still requires confirmation in the case of PF patients living in southeastern Brazil.

    Purpose: To georeference the distribution of Simuliidae (Diptera: Simuliidae) and Phlebotominae (Diptera: Psychodidae) and of PF cases in the northeastern region of São Paulo State, and to assess the humoral immune response against salivary gland extracts (SGEs) from biting flies in PF patients, relatives, and neighbours.

    Methods: PF patients' medical information recorded between 1965 and 2014 were obtained from the database of the University Hospital. Read More

    New Evidence Supporting Cyclosporine Efficacy in Epidermal Necrolysis.
    J Invest Dermatol 2017 Oct;137(10):2047-2049
    Paris, France (retired).
    Sixty years after its original description by Sir Alan Lyell, epidermal necrolysis (from Stevens-Johnson syndrome to toxic epidermal necrolysis) seems finally amenable to a specific treatment in addition to essential symptomatic measures in specialized settings. A recently published systematic review and an article by Gonzales-Herrada et al. strongly suggest that cyclosporine is effective in reducing the risk of death. Read More

    Developing biomarkers for predicting clinical relapse in pemphigus patients treated with rituximab.
    J Am Acad Dermatol 2017 Dec 18;77(6):1074-1082. Epub 2017 Sep 18.
    Department of Dermatology, Emory University School of Medicine, Atlanta, Georgia. Electronic address:
    Background: Rituximab is an effective therapy for pemphigus, although relapses are common.

    Objective: To identify biomarkers to predict relapse of pemphigus following rituximab treatment.

    Methods: In this retrospective cohort study, 62 patients with pemphigus treated with 99 rituximab cycles provided longitudinal clinical scoring and biomarker data, including levels of CD19B cells, CD4T cells, and desmoglein 1 (Dsg1) and desmoglein 3 (Dsg3) autoantibodies. Read More

    Minor Salivary Gland Transplantation for Severe Dry Eyes.
    Cornea 2017 Nov;36 Suppl 1:S26-S33
    *Department of Ophthalmology, Federal University of São Paulo, São Paulo, Brazil; and†Department of Pathology, LIM 14, São Paulo University School of Medicine, São Paulo, Brazil.
    Dry eye is a multifactorial disease comprising a wide spectrum of ocular surface alterations and symptoms of discomfort. In most patients with aqueous-deficient dry eye, pharmaceutical tear substitutes are used to control symptoms and prevent ocular surface damage. However, in severe dry eye conditions caused by cicatricial disorders, such as Stevens-Johnson syndrome and ocular cicatricial mucous membrane pemphigoid, noninvasive treatments are insufficient, and patients are at risk of developing complications that can lead to blindness. Read More

    Atypical features and systemic associations in extensive cases of Grover disease: A systematic review.
    J Am Acad Dermatol 2017 Nov 14;77(5):952-957.e1. Epub 2017 Sep 14.
    Department of Pathology, University of California, San Francisco, California. Electronic address:
    Background: Grover disease is an acantholytic disorder that typically occurs on the trunk of older individuals, primarily white men, in association with heat and xerosis. Cases with extensive and/or atypical distributions have been reported.

    Objective: To review the literature characterizing the population, morphology, associations, and disease course of extensive or atypical eruptions of Grover disease. Read More

    Oral mucosa biology and salivary biomarkers.
    Clin Dermatol 2017 Sep - Oct;35(5):477-483. Epub 2017 Jun 27.
    Department of Dermatology, University of California, Davis School of Medicine, Sacramento, CA. Electronic address:
    Although the surfaces of both the skin and oral mucosa are protected by squamous epithelial cells and fall within the scope of dermatologic practice, the oral cavity contains highly specialized structures and functions distinct from other skin biology and pathologic conditions and are also the purview of clinicians who care for patients with skin and mucosal diseases. We describe the distinct features of the tongue, mucosa, and salivary glands. In particular, we examine the composition and function of the saliva, with special focus on salivary biomarkers. Read More

    Successful Placement of a BAHA Implant in a Patient With Epidermolysis Bullosa: A Case Report and Review of the Literature.
    Ann Otol Rhinol Laryngol 2017 Nov 12;126(11):778-780. Epub 2017 Sep 12.
    5 Michigan Pediatric Ear, Nose, and Throat Associates, West Bloomfield, MI, USA.
    Introduction: Epidermolysis bullosa (EB) is a spectrum of mechanobullous disorders characterized by blistering following minor trauma or traction to the skin. Hearing loss in this population is poorly described in the otolaryngology literature, and its treatment oftentimes results in external auditory canal skin irritation.

    Case Presentation: We present the case of a 26-year-old female with EB and mixed hearing loss unable to wear conventional hearing aids due to sequelae of the external auditory canals. Read More

    Rapid generation of Col7a1mouse model of recessive dystrophic epidermolysis bullosa and partial rescue via immunosuppressive dermal mesenchymal stem cells.
    Lab Invest 2017 Oct 11;97(10):1218-1224. Epub 2017 Sep 11.
    Division of Blood and Marrow Transplantation, Department of Pediatrics, University of Minnesota Medical School, Minneapolis, MN, USA.
    Recessive dystrophic epidermolysis bullosa (RDEB) is a debilitating and ultimately lethal blistering disease caused by mutations to the Col7a1 gene. Development of novel cell therapies for the treatment of RDEB would be fostered by having immunodeficient mouse models able to accept human cell grafts; however, immunodeficient models of many genodermatoses such as RDEB are lacking. To overcome this limitation, we combined the clustered regularly interspaced short palindromic repeats and associated nuclease (CRISPR/Cas9) system with microinjection into NOD/SCID IL2rγc(NSG) embryos to rapidly develop an immunodeficient Col7a1mouse model of RDEB. Read More

    Autoimmune Progesterone Dermatitis Presenting as Stevens-Johnson Syndrome.
    Obstet Gynecol 2017 Oct;130(4):881-884
    Division of Reproductive Endocrinology and Infertility, Department of Obstetrics and Gynecology, Naval Medical Center San Diego, San Diego, California.
    Background: Autoimmune progesterone dermatitis is an uncommon disease presenting with cyclical skin eruptions corresponding with the menstrual cycle luteal phase. Because symptoms are precipitated by rising progesterone levels, treatment relies on hormone suppression.

    Case: A 22-year-old nulligravid woman presented with symptoms mistaken for Stevens-Johnson syndrome. Read More

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