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    1 OF 11

    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

    Lupus-like cutaneous reaction following pembrolizumab: An immune-related adverse event associated with anti-PD-1 therapy.
    J Cutan Pathol 2017 Oct 13. Epub 2017 Oct 13.
    Department of Dermatology, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania.
    PD-1 (programmed cell death-1) inhibitors, used to treat metastatic melanoma and other malignancies, are associated with development of immune-related adverse events in the skin. Such reactions include morbilliform eruptions, vitiligo, alopecia areata and bullous pemphigoid. In this report, we describe a patient who developed a lupus-like cutaneous reaction in the setting of pembrolizumab therapy for metastatic melanoma, adding to the spectrum of reactions which may be observed in association with PD-1 inhibitor therapy. Read More

    Bullous systemic lupus erythematosus in a 6-year-old boy.
    Pediatr Dermatol 2017 Nov 29;34(6):e313-e316. Epub 2017 Aug 29.
    Department of Dermatology, School of Medicine, Yale University, New Haven, CT, USA.
    Bullous systemic lupus erythematosus (BSLE) is a rare subepidermal blistering disorder characterized by an acute vesiculobullous eruption in a subset of individuals with systemic lupus erythematosus. BSLE most commonly affects young women and only rarely affects children. Herein we report a rare case of BSLE in a 6-year-old boy. Read More

    The Value of Direct Immunofluorescence on Proteinase-Digested Formalin-Fixed Paraffin-Embedded Skin Biopsies.
    Am J Dermatopathol 2017 Aug 9. Epub 2017 Aug 9.
    Department of Pathology, University of Massachusetts Medical School, UMass Memorial Medical Center, Worcester, MA.
    Direct immunofluorescence (DIF) on frozen tissue (DIF-F) is the method of choice for the identification of immune deposits present in skin and other tissues. DIF can also be performed on formalin-fixed paraffin-embedded tissue (DIF-P) after antigen retrieval with proteases and has proven to be of value in renal pathology. However, its utility in skin biopsies has not been fully examined. Read More

    Persistent lip enlargement: An unusual presentation of lupus erythematosus.
    Int J Womens Dermatol 2017 Jun 22;3(2):96-99. Epub 2016 Dec 22.
    Autoimmune Bullous Diseases Research Center, Department of Dermatology, Tehran University of Medical Sciences, Tehran, Iran.
    Macrocheilia is a challenging problem with a variety of underlying causes that are both local and systemic, and granulomatous causes underlie the majority of cases. In this study, we report on a 31-year old man who presented with a chronic lower lip enlargement and a nodular submental erythematous lesion. He was otherwise clinically healthy. Read More

    Unusual presentation of systemic lupus erythematosus.
    Saudi J Kidney Dis Transpl 2017 May-Jun;28(3):653-656
    Department of Nephrology, M. S. Ramaiah Memorial Hospital, Bengaluru, Karnataka, India.
    Bullous systemic lupus erythematosus is a rare distinctive subepidermal blistering disorder that can occur in patients with systemic lupus erythematosus (SLE). It is histologically characterized by a neutrophil-predominant infiltrate in the subepidermal region with deposition of immunoglobulins (IgG, IgA, IgM), C3, and auto-antibodies against collagen VII. Herein, we report a case of a 13-year-old girl who presented with bullous SLE and Class III lupus nephritis, which is extremely rare at this age. Read More

    Evaluation of prolactin levels in patients with newly diagnosed pemphigus vulgaris and its correlation with pemphigus disease area index.
    Int J Womens Dermatol 2016 Jun 23;2(2):53-55. Epub 2016 Apr 23.
    Department of Dermatology, Tehran University of Medical Sciences, Tehran, Iran.
    Background: Prolactin is a hormone; in addition to it known roles, it has immunomodulatory effects on lymphocytes maturation and immunoglobulins production. Hyperprolactinemia has been demonstrated in various autoimmune diseases such as systemic lupus erythematosus, rheumatoid arthritis, type I diabetes mellitus, and Graves' disease. In view of the prolactin immunomodulatory roles, studying prolactin levels in pemphigus as an autoimmune blistering disease may introduce new ways of understanding disease etiology and developing treatment strategies. Read More

    [Thymoma and autoimmune diseases].
    Rev Med Interne 2017 Mar 29. Epub 2017 Mar 29.
    Service de médecine interne, hospices civils de Lyon, hôpital de la Croix-Rousse, université Claude-Bernard - Lyon 1, 103, grande rue de la Croix-Rousse, 69004 Lyon, France.
    The association between thymoma and autoimmunity is well known. Besides myasthenia gravis, which is found in 15 to 20% of patients with thymoma, other autoimmune diseases have been reported: erythroblastopenia, systemic lupus erythematosus, inflammatory myopathies, thyroid disorders, Isaac's syndrome or Good's syndrome. More anecdotally, Morvan's syndrome, limbic encephalitis, other autoimmune cytopenias, autoimmune hepatitis, and bullous skin diseases (pemphigus, lichen) have been reported. Read More

    A 12-year retrospective review of bullous systemic lupus erythematosus in cutaneous and systemic lupus erythematosus patients.
    Lupus 2017 Oct 30;26(12):1278-1284. Epub 2017 Mar 30.
    Division of Dermatology, Department of Internal Medicine, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand.
    Objective The aim of this study was to investigate the clinical features, laboratory findings, systemic manifestations, treatment and outcome of patients with bullous systemic lupus erythematosus in a tertiary care center in Thailand. Methods We performed a retrospective review from 2002 to 2014 of all patients who fulfilled the diagnostic criteria for bullous systemic lupus erythematosus to evaluate for the clinical characteristics, extracutaneous involvement, histopathologic features, immunofluorescence pattern, serological abnormalities, internal organ involvement, treatments and outcome. Results Among 5149 patients with cutaneous lupus erythematosus and/or systemic lupus erythematosus, 15 developed vesiculobullous lesions. Read More

    Systemic Lupus Erythematosus and Bullous Pemphigoid with Dramatic Response to Dapsone.
    Am J Case Rep 2017 Mar 29;18:317-319. Epub 2017 Mar 29.
    NEUROFARBA Department, University of Florence and AOU Meyer, Florence, Italy.
    BACKGROUND Bullous pemphigoid is an autoimmune blistering disease, with relapses, isolated or associated with other autoimmune diseases such as systemic lupus erythematosus (SLE). Joint manifestations rapidly respond to small or moderate doses of corticosteroids, whereas skin manifestations usually respond to antimalarial drugs. CASE REPORT We describe the clinical case of an 11-year-old girl with SLE. Read More

    TWEAK/Fn14 Activation Contributes to the Pathogenesis of Bullous Pemphigoid.
    J Invest Dermatol 2017 Jul 27;137(7):1512-1522. Epub 2017 Mar 27.
    Department of Dermatology, The Second Affiliated Hospital, School of Medicine, Xi'an Jiaotong University, Xi'an, China. Electronic address:
    TWEAK participates in various cellular effects by engaging its receptor of Fn14. Increased levels of soluble TWEAK are associated with systemic autoimmunity in patients with lupus erythematosus, rheumatoid arthritis, or dermatomyositis. However, the role of TWEAK in bullous pemphigoid (BP) remains unknown. Read More

    Common Skin Conditions in Children: Noninfectious Rashes.
    FP Essent 2017 Feb;453:18-25
    University of North Carolina Chapel Hill School of Medicine Dermatology Residency Program, 410 Market St. Suite 400 CB#7715, Chapel Hill, NC 27516.
    Cutaneous adverse drug reactions are among the most common noninfectious rashes of childhood. Cutaneous adverse drug reactions are classified as morbilliform, urticarial, bullous, pustular, or psoriasiform. Atopic dermatitis is one of the most common inflammatory cutaneous eruptions, and is characterized by pruritus and flexural distribution. Read More

    Pathogenic mechanisms of IgE-mediated inflammation in self-destructive autoimmune responses.
    Autoimmunity 2017 Feb;50(1):25-36
    a Department of Respiratory , Inflammation & Autoimmunity, MedImmune LLC , Gaithersburg , MD , USA.
    Autoantibodies of the IgG subclass are pathogenic in a number of autoimmune disorders such as systemic lupus erythomatosus. The presence of circulating IgE autoantibodies in autoimmune patients has also been known for almost 40 years. Despite their role in allergies, IgE autoantibodies are not associated with a higher rate of atopy in these patients. Read More

    Bullous Systemic Lupus Erythematosus and Lupus Nephritis in a Young Girl.
    Oman Med J 2016 Nov;31(6):453-455
    Division of Pediatric Nephrology, Child Growth and Development Research Center, Research Institute for Primordial Prevention of Non-Communicable Disease, Isfahan University of Medical Sciences Isfahan, Iran.
    Bullous systemic lupus erythematosus (BSLE) is an autoimmune blistering disease occurring in patients with systemic lupus erythematosus (SLE). It is a rare disease, especially in children. A 14-year-old girl initially presented with fatigue, generalized vesiculobullous skin lesions, and ulcers over the hard palate and oral mucosa. Read More

    Diffuse Bullous Eruptions in an Elderly Woman: Late-Onset Bullous Systemic Lupus Erythematosus.
    Case Rep Dermatol 2016 Sep-Dec;8(3):278-282. Epub 2016 Oct 13.
    Department of Internal Medicine, Advocate Illinois Masonic Medical Center, Chicago, Ill., USA.
    Vesiculobullous eruptions in the elderly represent a diverse range of varying pathophysiologies and can present a significant clinical dilemma to the diagnostician. Diagnosis requires a careful review of clinical history, attention to detail on physical and histomorphological examination, and appropriate immunofluorescence testing. We describe the case of a 73-year-old female who presented to our hospital with a painful blistering skin rash developed over 2 days. Read More

    [Systemic lupus erythematosus : Unusual cutaneous manifestations].
    Hautarzt 2016 Dec;67(12):970-981
    Abteilung Dermatologie und Venerologie, Krankenanstalt Rudolfstiftung, Juchgasse 25, 1030, Wien, Österreich.
    Background: Various different mucocutaneous symptoms may affect up to 80 % of systemic lupus erythematosus (SLE) patients.

    Objectives: To investigate, various unspecific, but otherwise typical clinical symptoms of skin and mucous membranes that arise in SLE patients other than those defined as SLE criteria such as butterfly rash, chronic cutaneous lupus erythematosus, oral ulcers, and increased photosensitivity.

    Materials And Methods: Extensive search of peer-reviewed scientific articles was performed, medical histories of several SLE patients seen in our department were analyzed, and the rare disease courses in three SLE patients are presented. Read More

    Lenalidomide for refractory cutaneous manifestations of pediatric systemic lupus erythematosus.
    Lupus 2017 May 12;26(6):646-649. Epub 2016 Nov 12.
    2 Department of Pediatrics, Duke University Medical Center, Durham, USA.
    Objective Cutaneous manifestations of pediatric systemic lupus erythematosus cause significant morbidity. Lenalidomide, a thalidomide analogue, has shown promise treating cutaneous lupus erythematosus in adults. Our objective was to evaluate lenalidomide's efficacy and safety in treating refractory cutaneous manifestations of pediatric systemic lupus erythematosus. Read More

    Case of Pediatric Bullous Systemic Lupus Erythematosus Treated with Intravenous Immunoglobulin.
    Pediatr Dermatol 2017 Jan 25;34(1):e54-e56. Epub 2016 Oct 25.
    Department of Dermatology, Icahn School of Medicine at Mount Sinai, New York, New York.
    This is a case report of a 16-year-old girl recently diagnosed with systemic lupus erythematosus (SLE) who presented with multiple blisters on the face, hands, arms, legs, trunk, and vaginal and oral mucosa. Skin biopsy was consistent with bullous SLE (BSLE). Dapsone is often the first-line treatment option for BSLE, but the patient's history of anemia and leukopenia and long-term immunosuppression requirement for her systemic symptoms raised concerns about dapsone and bone marrow toxicity, especially hemolytic anemia and agranulocytosis. Read More

    Decreased PD-1 positive blood follicular helper T cells in patients with psoriasis.
    Arch Dermatol Res 2016 Oct 8;308(8):593-9. Epub 2016 Aug 8.
    Department of Dermatology, Severance Hospital, Cutaneous Biology Research Institute, 50-1 Yonsei-Ro, Seodaemun-gu, Seoul, 120-752, Korea.
    Follicular helper T (Tfh) cells are recently characterized subset of helper T cells, which are initially found in the germinal centers of B cell follicles. The major role of Tfh cells is helping B cell activation and antibody production during humoral immunity. Recently, blood Tfh cells were shown to be associated with autoimmune diseases, such as systemic lupus erythematosus, rheumatoid arthritis, bullous pemphigoid and psoriasis. Read More

    [Terbinafine : Drug-induced lupus erythematodes and triggering of psoriatic skin lesions].
    Hautarzt 2016 Sep;67(9):724-31
    , Hofmannstr. 11, 35444, Biebertal, Deutschland.
    Based on the technical information that oral terbinafine must be used with caution in patients with pre-existing psoriasis or lupus erythematosus, the literature was summarized. Terbinafine belongs to the drugs able to induce subcutaneous lupus erythematosus (SCLE)-with a relatively high risk. The clinical picture of terbinafine-induced SCLE may be highly variable and can also include erythema exsudativum multiforme-like or bullous lesions. Read More

    The incidence of herpes zoster in cutaneous lupus erythematosus (CLE), dermatomyositis (DM), pemphigus vulgaris (PV), and bullous pemphigoid (BP).
    J Am Acad Dermatol 2016 Jul;75(1):42-8
    Corporal Michael J. Crescenz Department of Veterans Affairs Medical Center, Philadelphia, Pennsylvania; Department of Dermatology, University of Pennsylvania, Philadelphia, Pennsylvania. Electronic address:
    Background: Herpes zoster is a common condition that causes significant morbidity.

    Objective: This study determined the incidence of zoster in patients with cutaneous lupus erythematosus (CLE), dermatomyositis (DM), pemphigus vulgaris (PV), and bullous pemphigoid (BP).

    Methods: In this retrospective cohort study the electronic medical records of 186 patients with CLE, 103 with DM, 83 with PV, 44 with BP, and 152 healthy control patients were reviewed to confirm positive diagnoses of zoster. Read More

    Role of IgE in autoimmunity.
    J Allergy Clin Immunol 2016 Jun 27;137(6):1651-1661. Epub 2016 Apr 27.
    Respiratory, Inflammation & Autoimmunity Department, Research, MedImmune, Gaithersburg, Md.
    There is accumulating evidence to suggest that IgE plays a significant role in autoimmunity. The presence of circulating self-reactive IgE in patients with autoimmune disorders has been long known but, at the same time, largely understudied. However, studies have shown that the increased IgE concentration is not associated with higher prevalence for atopy and allergy in patients with autoimmune diseases, such as systemic lupus erythematosus. Read More

    Toxic Epidermal Necrolysis-Like Cutaneous Lupus in Pediatric Patients: A Case Series and Review.
    Pediatrics 2016 Jun;137(6)
    Division of Pediatric Dermatology, Departments of Dermatology and Pediatrics, Medical College of Wisconsin, Milwaukee, Wisconsin;
    Bullous eruptions in patients with underlying systemic lupus erythematosus (LE) can mimic toxic-epidermal necrolysis (TEN), a rapidly progressive mucocutaneous reaction usually associated with medication use. Differentiating between classic drug-induced TEN and TEN-like cutaneous LE is important but difficult. We report a series of 3 patients with pediatric systemic LE who were admitted with severe worsening of skin disease resembling TEN. Read More

    Utility of Direct Immunofluorescence Studies in Subclassification of Autoimmune Sub-Epidermal Bullous Diseases: A 2-Year Study in a Tertiary Care Hospital.
    Turk Patoloji Derg 2016 ;32(2):91-8
    Department of Pathology Bharati Vidyapeeth Deemed University Medical College and Hospital, SANGLI, INDIA.
    Objective: Sub-epidermal bullous disorders belong to immunobullous diseases which develop as a result of autoantibody action against epidermal basement membrane proteins. Clinically, they are tense bullae and do not rupture easily. They are classified into various forms based on histopathology and direct immunofluorescence patterns. Read More

    Periodic Acid-Schiff Staining Parallels the Immunoreactivity Seen By Direct Immunofluorescence in Autoimmune Skin Diseases.
    N Am J Med Sci 2016 Mar;8(3):151-5
    Department of Dermatopathology, Georgia Dermatopathology Associates, Atlanta, Georgia, USA.
    Background: In many countries and laboratories, techniques such as direct immunofluorescence (DIF) are not available for the diagnosis of skin diseases. Thus, these laboratories are limited in the full diagnoses of autoimmune skin diseases, vasculitis, and rheumatologic diseases. In our experience with these diseases and the patient's skin biopsies, we have noted a positive correlation between periodic acid-Schiff (PAS) staining and immunofluorescence patterns; however, these were just empiric observations. Read More

    Clinical, demographic and immunopathological spectrum of subepidermal autoimmune bullous diseases at a tertiary center: A 1-year audit.
    Indian J Dermatol Venereol Leprol 2016 May-Jun;82(3):358
    Department of Dermatology, Venereology and Leprology, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
    Background: The subepidermal autoimmune bullous diseases are a subset of immunobullous diseases encountered less frequently in the Indian population. There is a paucity of data on the prevalence, demographic and clinicopathological spectrum of various subepidermal autoimmune bullous diseases from India.

    Aim: To determine the demographic and clinicopathological profile of subepidermal autoimmune bullous diseases in Indian patients, presenting to the Immunobullous Disease Clinic of Postgraduate Institute of Medical Education and Research, Chandigarh. Read More

    Neonatal Autoimmune Blistering Disease: A Systematic Review.
    Pediatr Dermatol 2016 Jul 18;33(4):367-74. Epub 2016 Apr 18.
    Department of Dermatology, St. George Hospital, Sydney, New South Wales, Australia.
    We aimed to better understand the pathogenesis, clinical features, prognosis, and treatment of neonatal autoimmune blistering diseases (AIBDs). We searched Medline, Embase, PubMed, Latin American and Caribbean Health Sciences Literature, and reference lists of identified articles. Inclusion criteria were articles published from 1946 to December 2014 in any language. Read More

    Validity of trichoscopy in the diagnosis of primary cicatricial alopecias.
    Int J Dermatol 2016 Oct 7;55(10):1106-14. Epub 2016 Apr 7.
    Department of Dermatology, Autoimmune Bullous Diseases Research Center, Tehran University of Medical Sciences, Tehran, Iran.
    Background: There are limited data on the validity of dermatoscopy in primary cicatricial alopecias (PCAs) and its subtypes, including lichen planopilaris and discoid lupus erythematosus. Trichoscopic features of PCAs, their prevalence, and validity were evaluated in this study.

    Methods: One hundred patients with PCA underwent dermatoscopy. Read More

    Sex differences in the incidence of skin and skin-related diseases in Olmsted County, Minnesota, United States, and a comparison with other rates published worldwide.
    Int J Dermatol 2016 Sep 24;55(9):939-55. Epub 2016 Mar 24.
    Division of Clinical Dermatology, Mayo Clinic, Rochester, MN, USA.
    Many skin and skin-related diseases affect the sexes unequally, with attendant implications for public health and resource allocation. To evaluate better the incidence of skin and skin-related diseases affecting males vs. females, we reviewed published population-based epidemiology studies of skin disorders performed utilizing Rochester Epidemiology Project data. Read More

    Herpes zoster infection in childhood-onset systemic lupus erythematosus patients: a large multicenter study.
    Lupus 2016 Jun 27;25(7):754-9. Epub 2016 Jan 27.
    Pediatric Rheumatology Unit, Faculdade de Medicina da Universidade de São Paulo, Brazil
    Objective: The aim of this multicenter study in a large childhood-onset systemic lupus erythematosus (cSLE) population was to assess the herpes zoster infection (HZI) prevalence, demographic data, clinical manifestations, laboratory findings, treatment, and outcome.

    Methods: A retrospective multicenter cohort study (Brazilian cSLE group) was performed in ten Pediatric Rheumatology services in São Paulo State, Brazil, and included 852 cSLE patients. HZI was defined according to the presence of acute vesicular-bullous lesions on erythematous/edematous base, in a dermatomal distribution. Read More

    Blisters and Loss of Epidermis in Patients With Lupus Erythematosus: A Clinicopathological Study of 22 Patients.
    Medicine (Baltimore) 2015 Nov;94(46):e2102
    From the Faculté de Médecine, Université de Strasbourg; Clinique Dermatologique, Hôpitaux Universitaires de Strasbourg, Strasbourg (CM-D, DL); Faculté de Médecine, Université de Montpellier; Département de Dermatologie, Hôpital Saint Eloi, CHRU Montpellier, Montpellier (DB); Faculté de Médecine, Université Pierre-et-Marie-Curie Paris-VI, Service de Dermatologie Hôpital Tenon, Paris (CF); Faculté de Médecine, Université Claude Bernard Lyon 1, Service de Dermatologie, CHU Lyon Sud, Pierre-Bénite (NP, SD); and Service de Dermatologie-Médecine interne, CHU de Pointe-à-Pitre, Pointe-à-Pitre, Guadeloupe, (NC) France.
    The nosology of bullous lesions or equivalents (vesicles, erosions, and crusts) in patients with lupus erythematosus (LE) is rarely addressed.The primary aim of this study was to draw up a precise phenotypic inventory of such skin lesions; the secondary objective was to assess a potential relationship between the different types of loss of epidermis and extracutaneous lupus manifestations.We conducted a retrospective multicenter study including 22 patients with definite LE and bullous lesions or equivalents. Read More

    [Bullous systemic lupus mimicking a Stevens-Johnson syndrome].
    Rev Alerg Mex 2015 Oct-Dec;62(4):323-7
    Unidad de Dermatología, Fundación Valle de Lili, Cali, Colombia.
    Autoimmune bullous diseases represent a diagnostic challenge due to the wide spectrum of pathologies that share similar clinical features. This paper reports the case of a woman admitted with a supposed diagnosis of a Stevens-Johnson syndrome, in which the history, the profile of autoimmunity and interdisciplinary approach were of vital importance to clarify the clinical picture. Read More

    Immunofluorescence profile of discoid lupus erythematosus.
    Indian J Pathol Microbiol 2015 Oct-Dec;58(4):479-82
    Department of Immunopathology, PGIMER, Chandigarh, India.
    The direct immunofluorescence (DIF) of skin in conjunction with histopathology gives the best diagnostic yield. It is invaluable in confirming the diagnosis of small vessel vasculitides and bullous lesions of the skin and can be used as an additional tool to pinpoint the diagnosis of systemic and localized autoimmune diseases involving the skin. This study was undertaken to analyze the strength of DIF vis-à -vis histopathology in the diagnosis of discoid lupus erythematosus (DLE) and at the same time to elaborate the specific immunofluorescence findings in the lesions of DLE. Read More

    Autoimmune myelofibrosis with pancytopenia as a presenting manifestation of systemic lupus erythematosus responsive to mycophenolate mofetil.
    Lupus 2016 Apr 3;25(4):427-30. Epub 2015 Nov 3.
    Division of Rheumatology, Mayo Clinic, Rochester, USA.
    Hematological abnormalities, such as anemia, leucopenia, and thrombocytopenia, secondary to peripheral destruction, are common in systemic lupus erythematosus (SLE). However, cytopenias from autoimmune myelofibrosis (AIMF) are extremely uncommon in SLE, with less than 40 reported cases in the literature. We report the case of a 33-year-old female who presented with bullous skin lesions and pancytopenia as the presenting manifestation of what was ultimately diagnosed as SLE with AIMF. Read More

    Retrospective Study on Autoimmune Blistering Disease in Paediatric Patients.
    Pediatr Dermatol 2015 Nov-Dec;32(6):845-52. Epub 2015 Sep 22.
    Division of Dermatology, National University Hospital, Singapore, Singapore.
    Background: Autoimmune blistering diseases (AIBDs) are rare in children and their prevalence in Singapore is unclear. We aimed to investigate the clinical and immunopathologic characteristics of children diagnosed with AIBDs in Singapore.

    Materials And Methods: The clinical and histology databases at the National Skin Centre in Singapore were searched to identify patients younger than 18 years old diagnosed with an AIBD from January 1, 1998, through December 31, 2012. Read More

    Two cases of annular erythema without bullous lesions by autoimmune blistering diseases.
    Mod Rheumatol 2015 Oct 19:1-3. Epub 2015 Oct 19.
    a Department of Dermatology , Shiga University of Medical Science , Setatsukinowa , Otsu , Shiga , Japan.
    Although annular erythema is usually observed as one of the cutaneous manifestations of Sjögren's syndrome or subacute cutaneous lupus erythematosus, autoimmune blistering diseases also present with annular erythema. However, bullous lesions are not always found, and there is a rare type without bullous lesions. We present two cases of autoimmune blistering diseases showing annular erythema without bullous lesions. Read More

    Update on lichen planus and its clinical variants.
    Int J Womens Dermatol 2015 Aug 16;1(3):140-149. Epub 2015 Sep 16.
    Department of Dermatology, University of Connecticut Health Center, Farmington, CT.
    Lichen planus (LP) is an inflammatory skin condition with characteristic clinical and histopathological findings. Classic LP typically presents as pruritic, polygonal, violaceous flat-topped papules and plaques; many variants in morphology and location also exist, including oral, nail, linear, annular, atrophic, hypertrophic, inverse, eruptive, bullous, ulcerative, lichen planus pigmentosus, lichen planopilaris, vulvovaginal, actinic, lichen planus-lupus erythematosus overlap syndrome, and lichen planus pemphigoides. Clinical presentation of the rarer variant lesions may be largely dissimilar to classic LP and therefore difficult to diagnose based solely on clinical examination. Read More

    Treatment of Bullous Systemic Lupus Erythematosus.
    J Immunol Res 2015 18;2015:167064. Epub 2015 May 18.
    Department of Rheumatology and Clinical Immunology, The First Hospital of Xiamen University, Xiamen 361003, China.
    Bullous systemic lupus erythematosus (BSLE) is an autoantibody-mediated vesiculobullous disease in patients with SLE. Autoimmunity in BSLE is characterized by the presence of circulating anti-type VII collagen antibodies. BSLE patients often present with multiple, tense, clear fluid-filled vesicles and bullae overlying erythematous edematous plaques. Read More

    Comparative Study of Direct Immunofluorescence in Discoid Lupus Erythematosus and Bullous Pemphigoid.
    Am J Dermatopathol 2016 Feb;38(2):121-3
    Department of Dermatology, Kurume University School of Medicine, Kurume, Japan.
    Immunoreactants are found in the epidermal basement membrane in both lupus erythematosus and bullous pemphigoid (BP). To our knowledge, there are no comparative studies on direct immunofluorescence (DIF) of discoid lupus erythematosus (DLE) and BP. The authors studied DIF of lesional skins in 9 patients (2 males and 7 females) with DLE and 29 patients (11 males and 18 females) with BP to disclose the difference between these 2 diseases. Read More

    Allelic and copy-number variations of FcγRs affect granulocyte function and susceptibility for autoimmune blistering diseases.
    J Autoimmun 2015 Jul 29;61:36-44. Epub 2015 May 29.
    Institute of Experimental Dermatology, University of Lübeck, Lübeck, Germany. Electronic address:
    Low-affinity Fcγ receptors (FcγR) bridge innate and adaptive immune responses. In many autoimmune diseases, these receptors act as key mediators of the pathogenic effects of autoantibodies. Genes encoding FcγR exhibit frequent variations in sequence and gene copy number that influence their functional properties. Read More

    Role of direct immunofluorescence in dermatological disorders.
    Indian Dermatol Online J 2015 May-Jun;6(3):172-80
    Department of Dermatology, M. S. Ramaiah Medical College and Hospital, Bengaluru, Karnataka, India.
    Background: Direct immunofluorescence (DIF) test for tissue-bound autoantibodies, has been found to be of value in the diagnosis of several dermatological disorders. The location and pattern of deposition of immunoreactants helps in classifying various immune-mediated diseases.

    Aims And Objectives: The aim of this study was to analyze the concordance between the clinical, histopathological and DIF diagnosis in bullous and nonbullous lesions of the skin, and thus determine the impact of immunofluorescence on diagnosis. Read More

    Heat shock protein 90: a pathophysiological factor and novel treatment target in autoimmune bullous skin diseases.
    Exp Dermatol 2015 Aug 3;24(8):567-71. Epub 2015 Jun 3.
    Department of Dermatology, University of Lübeck, Lübeck, Germany.
    The chaperone heat shock protein 90 (Hsp90), a cell stress-inducible molecule that regulates activity of many client proteins responsible for cellular growth, differentiation and apoptosis, has been proposed as an important therapeutic target in patients with malignancies. More recently, its active participation in (auto)immune processes has been recognized as evidenced by amelioration of inflammatory disease pathways through pharmacological inhibition of Hsp90 in rodent models of autoimmune encephalomyelitis, rheumatoid arthritis and systemic lupus erythematosus. Based on own current research results, this viewpoint essay provides important insights that Hsp90 is also involved as a notable pathophysiological factor in autoimmune blistering dermatoses including epidermolysis bullosa acquisita, bullous pemphigoid and possibly dermatitis herpetiformis. Read More

    Bullous systemic lupus erythematosus associated with esophagitis dissecans superficialis.
    Case Rep Rheumatol 2015 2;2015:930683. Epub 2015 Mar 2.
    Department of Medicine, Interfaith Medical Center, Brooklyn, NY 11213, USA.
    Bullous systemic lupus erythematosus is one of the rare autoantibody mediated skin manifestation of systemic lupus erythematosus (SLE) demonstrating subepidermal blistering with neutrophilic infiltrate histologically. We present a case of a 40-year-old Hispanic female who presented with a several months' history of multiple blistering pruritic skin lesions involving the face and trunk, a photosensitive rash over the face and neck, swelling of the right neck lymph node, and joint pain involving her elbows and wrist. Her malady was diagnosed as bullous systemic lupus erythematosus based on the immunological workup and biopsy of her skin lesions. Read More

    Complement system in dermatological diseases - fire under the skin.
    Front Med (Lausanne) 2015 29;2. Epub 2015 Jan 29.
    Department of Bacteriology and Immunology, Haartman Institute, University of Helsinki , Helsinki , Finland ; Huslab, Helsinki University Central Hospital , Helsinki , Finland ; Research Programs Unit, Immunobiology, University of Helsinki , Helsinki , Finland.
    The complement system plays a key role in several dermatological diseases. Overactivation, deficiency, or abnormality of the control proteins are often related to a skin disease. Autoimmune mechanisms with autoantibodies and a cytotoxic effect of the complement membrane attack complex on epidermal or vascular cells can cause direct tissue damage and inflammation, e. Read More

    Detection of Type VII Collagen Autoantibodies Before the Onset of Bullous Systemic Lupus Erythematosus.
    JAMA Dermatol 2015 May;151(5):539-43
    Department of Dermatology, University of Texas Southwestern Medical Center, Dallas.
    Importance: Anti-type VII collagen autoantibodies are often detectable in patients with bullous systemic lupus erythematosus (BSLE). However, the timing of their appearance preceding the onset of disease is unknown to date.

    Observations: We report the case of a 50-year-old woman with a history of SLE who was seen with vesicles and bullae around her lips, trunk, axillae, arms, and thighs. Read More

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