Clin Lab Med 2017 Sep;37(3):673-696

Department of Pathology, University of Michigan, 1301 Catherine Street, Medical Science I, M3261, Ann Arbor, MI 48109, USA; Department of Dermatology, University of Michigan, 1500 East Medical Center Drive, Ann Arbor, MI 48109, USA. Electronic address:

Owing to the wide variety and complexity of inflammatory skin diseases, inflammatory dermatopathology can be a challenging topic for dermatopathologists and general surgical pathologists alike. Following a basic tissue reaction pattern approach, this article reviews the most common and important entities of each pattern, with emphasis on differential diagnosis, diagnostic pitfalls, and appropriate workup when indicated. A few dermatologic emergencies are also discussed. Read More

Expert Rev Clin Pharmacol 2017 Jul 27:1-10. Epub 2017 Jul 27.

b UOC Dermatologia, IRCCS Fondazione Ca' Granda Ospedale Maggiore Policlinico, Dipartimento di Fisiopatologia Medico-Chirurgica e dei Trapianti , Università Degli Studi di Milano , Milan , Italy.

Introduction: Neutrophilic dermatoses are a heterogenous group of chronic, cutaneous inflammatory conditions characterized by the accumulation of neutrophils in the skin and by systemic inflammation. Neutrophilic dermatoses can be idiopathic or associated with other inflammatory or systemic diseases, including the group of the hereditary, autoinflammatory syndromes. Clinical management is challenging, due to limited clinical evidence and lack of clinical practice guidelines. Read More

Case Rep Dermatol 2017 Jan-Apr;9(1):140-144. Epub 2017 Apr 27.

aDepartment of Dermatology, University Hospital of Zurich, Zurich, Switzerland.

Subcorneal pustular dermatosis (SCPD, Sneddon-Wilkinson disease) is a rare chronic-relapsing skin disorder that typically manifests as flaccid sterile pustules without systemic symptoms. Although the accumulation of neutrophils is acknowledged to be a hallmark of SCPD, its exact pathomechanism is still not known. Several chemotactic factors have been implicated in neutrophil recruitment and invasion, including the proinflammatory cytokine TNF-α. Read More

Pan Afr Med J 2017 13;26:136. Epub 2017 Mar 13.

Université de Sousse, Tunisie.

Cicatricial Pemphigoid is a subepithelial bullous dermatosis which essentially involves the mucous membranes with cicatricial evolution We report the case of a 66-year old patient hospitalized with erosive gingivitis associated with dysphagia, dyspnea and blurred vision. Dermatologic examination showed erosive lesions involving the palate and the pharynx. Ophthalmologic examination showed symblepharons, ectropion and bilateral cataract. Read More

Nat Rev Dis Primers 2017 May 11;3:17026. Epub 2017 May 11.

Department of Dermatology, Keio University School of Medicine, 35 Shinanomachi, Shinjuku-ku, Tokyo 160-8582, Japan.

Pemphigus is a group of IgG-mediated autoimmune diseases of stratified squamous epithelia, such as the skin and oral mucosa, in which acantholysis (the loss of cell adhesion) causes blisters and erosions. Pemphigus has three major subtypes: pemphigus vulgaris, pemphigus foliaceus and paraneoplastic pemphigus. IgG autoantibodies are characteristically raised against desmoglein 1 and desmoglein 3, which are cell-cell adhesion molecules found in desmosomes. Read More

Neurology 2017 Jun 5;88(23):2183-2191. Epub 2017 May 5.

Author affiliations are provided at the end of the article.

Objective: To investigate the involvement of human leukocyte antigen (HLA) loci in aromatic antiepileptic drug-induced cutaneous adverse reactions.

Methods: A case-control study was performed to detect HLA loci involved in aromatic antiepileptic drug-induced Stevens-Johnson syndrome in a southern Han Chinese population. Between January 1, 2006, and December 31, 2015, 91 cases of Stevens-Johnson syndrome induced by aromatic antiepileptic drugs and 322 matched drug-tolerant controls were enrolled from 8 centers. Read More

Bratisl Lek Listy 2017 ;118(4):243-249

Aims: To determine the diagnostic accordance between histopathological and direct immunofluorescence diagnosis of patients with autoimmune vesiculobullous skin diseases.

Background: The term pemphigus refers to a group of autoimmune blistering diseases mediated by auto-antibodies directed against desmoglein proteins. The differentiation between the various bullous diseases is important for treatment and prognosis. Read More

Clin Toxicol (Phila) 2017 Sep 2;55(8):939-940. Epub 2017 May 2.

a Department of Pharmacy , Anning Branch Lanzhou General Hospital Lanzhou Command , Lanzhou , Gansu , P.R. China.

Iodixanol is a non-ionic contrast medium for general vascular use. The most common adverse effects from iodixanol include skin rashes, hives, erythema, itching, and angioedema. To date, there have been no reports of delayed genital blisters of iodixanol. Read More

Invest Ophthalmol Vis Sci 2017 Apr;58(4):2421-2429

Department of Ophthalmology, Faculty of Life Sciences, Kumamoto University, Kumamoto, Japan.

Purpose: Suberoylanilide hydroxamic acid (SAHA) has been shown to support the maintenance of experimental filtration blebs in animal models. This study was performed to investigate the molecular mechanisms underlying the bleb-maintaining effects of SAHA in modulating wound healing activities of conjunctival fibroblasts.

Methods: Human conjunctival fibroblasts (HConFs) were pretreated with SAHA before treatment with TGF-β2. Read More

Pediatr Infect Dis J 2017 May;36(5):513-515

From the Department of Dermatology, Dokumentationszentrum schwerer Hautreaktionen (dZh), Medical Center-University of Freiburg, Hauptstrasse 7, 79104 Freiburg, Germany.

Fever is a symptom that often accompanies skin eruptions, especially in children. It can be a sign of an infectious condition presenting with exanthems or it may precede an exanthematous eruption. Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are severe reactions affecting skin and mucosa with blisters and erosions. Read More

Yonsei Med J 2017 May;58(3):658-661

Department of Ophthalmology, Ajou University School of Medicine, Suwon, Korea.

When vitrectomy is performed in eyes that have undergone glaucoma surgery, the site of sclerotomy often overlaps with the previous glaucoma operation site. It can lead to serious complications such as postoperative hypotony, leakage, and/or infection. Our technique involves modification of surgeon's position and two sclerotomy sites 45° away from the original position, with an infusion cannula inserted infranasally to avoid damage to the glaucoma drainage implant or filtering bleb. Read More

JAMA Dermatol 2017 Jun;153(6):514-522

Dokumentationszentrum schwerer Hautreaktionen, Department of Dermatology, Medical Center - University of Freiburg, Faculty of Medicine, University of Freiburg, Freiburg, Germany.

Importance: Stevens-Johnson syndrome and toxic epidermal necrolysis (SJS/TEN) are rare but severe adverse reactions with high mortality. There is no evidence-based treatment, but various systemic immunomodulating therapies are used.

Objectives: To provide an overview on possible immunomodulating treatments for SJS/TEN and estimate their effects on mortality compared with supportive care. Read More

JAMA 2017 Mar;317(11):1169-1170

Center for Clinical Studies, Webster, Texas2Department of Dermatology, University of Texas Health Science Center at Houston.

Mediators Inflamm 2017 22;2017:7151285. Epub 2017 Feb 22.

Laboratory of Immunology, Federal University of Triângulo Mineiro, Uberaba, MG, Brazil.

Pemphigus vulgaris (PV) is an autoimmune disease characterized by the presence of IgG autoantibodies against desmoglein-3. Despite the variety of findings, the chemokine and cytokine profiles that characterize the immune response in the disease are still poorly explored. Thus, 20 PV patients and 20 controls were grouped according to gender, ethnicity, place of residence, and clinical parameters of the disease. Read More

Zhonghua Yan Ke Za Zhi 2017 Mar;53(3):177-181

Department of Ophthalmology, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing 100730, China.

Objective: To describe corneal alterations visible on in vivo confocal microscopy in patients with debilitating ocular sequelae caused by Stevens-Johnson syndrome (SJS) or toxic epidermal necrolysis (TEN). Methods: Twenty-two eyes of 11 consecutive patients suffering from chronic SJS or TEN were studied using slit lamp and in vivo confocal microscopy in the Ophthalmology Outpatient Department of Peking Union Medical College Hospital from March 2014 to April 2015. Results: Dry eye with meibomian gland dysfunction (19 eyes of 10 patients, 86. Read More

An Bras Dermatol 2016 Sep-Oct;91(5 suppl 1):81-83

Clinical Center of Serbia, Faculty of Medicine, University of Belgrade - Belgrade, Serbia.

Lichen sclerosus et atrophicus is a chronic disease of unknown etiology characterized by atrophic and sclerotic plaques in both genital and extragenital regions. Extensive bullous lichen sclerosus et atrophicus (BLSA) is a severe variant of the disease with no widely accepted treatment. We present a 63-year-old woman with extensive extragenital, ivory-colored, atrophic plaques on her trunk and extremities and disseminated hemorrhagic bullae. Read More

An Bras Dermatol 2016 Sep-Oct;91(5 suppl 1):35-38

Universidade Federal de São Paulo (Unifesp) - São Paulo (SP), Brazil.

Linear IgA dermatosis is a rare subepidermal autoimmune blistering disease characterized by linear deposition of IgA along the basement membrane zone. In the last three decades, many different drugs have been associated with the drug-induced form of the disease, especially vancomycin. We report a case of vancomycin-induced linear IgA disease mimicking toxic epidermal necrolysis. Read More

An Bras Dermatol 2016 Sep-Oct;91(5 suppl 1):32-34

Hospital Federal de Bonsucesso (HFB) -Rio de Janeiro (RJ), Brazil.

Childhood linear immunoglobulin A dermatosis is a rare autoimmune vesiculobullous disease. It results in linear deposition of autoantibodies (immunoglobulin A) against antigens in the basal membrane zone, leading to subepidermal cleavage. Additional depositions of immunoglobulin G and complement-3 might occur. Read More

JAMA Dermatol 2017 Jun;153(6):587-592

Department of Dermatology, University of California, Davis, Sacramento.

Importance: Toxic epidermal necrolysis (TEN) and Stevens-Johnson Syndrome (SJS) are rare, acute, life-threatening dermatologic disorders involving the skin and mucous membranes. Research into these conditions is hampered by a lack of standardization of case reporting and data collection.

Objective: To establish a standardized case report form to facilitate comparisons and maintain data quality based on an international panel of SJS/TEN experts who performed a Delphi consensus-building exercise. Read More

J Am Acad Dermatol 2017 May 9;76(5):811-817.e4. Epub 2017 Mar 9.

Department of Dermatology, Pediatrics, Northwestern University Feinberg School of Medicine, Chicago, Illinois; Department of Dermatology, Northwestern University Feinberg School of Medicine, Chicago, Illinois. Electronic address:

Background: Little is known about the epidemiology of Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) in children.

Objective: We sought to determine the morbidity, mortality, and comorbid health conditions of SJS and TEN in US children.

Methods: This was a cross-sectional study of the 2009 to 2012 Nationwide Inpatient Sample, which contains a representative 20% sample of all US hospitalizations. Read More

JAMA Ophthalmol 2017 Apr;135(4):355-360

Department of Frontier Medical Science and Technology for Ophthalmology, Kyoto, Prefectural University of Medicine, Kyoto, Japan.

Importance: Describing the association with human leukocyte antigen (HLA) alleles could facilitate the understanding of increased risk factors for development of Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) in patients with severe ocular complications (SOCs).

Objective: To investigate the association between HLA class I genes and cold medicine (CM)-associated SJS/TEN with SOCs.

Design, Setting, And Participants: This case-control study was conducted between February 8, 2013, and August 29, 2014. Read More

Zhonghua Er Ke Za Zhi 2017 Mar;55(3):223-224

J Am Acad Dermatol 2017 May 1;76(5):847-855.e5. Epub 2017 Mar 1.

Department of Medicine, Division of Dermatology, University of Massachusetts Medical School, Worcester, Massachusetts. Electronic address:

Background: Vitiligo is an autoimmune disease of the skin with limited treatment options; there is an urgent need to identify and validate biomarkers of disease activity to support vitiligo clinical studies.

Objective: To investigate potential biomarkers of disease activity directly in the skin of vitiligo subjects and healthy subjects.

Methods: Patient skin was sampled via a modified suction-blister technique, allowing for minimally invasive, objective assessment of cytokines and T-cell infiltrates in the interstitial skin fluid. Read More

J Dermatol 2017 Mar;44(3):232-242

Department of Dermatology and Section of Dermatopathology, Boston University School of Medicine, Boston, Massachusetts, USA.

Acantholysis is a commonly encountered histological pattern which typically generates a differential of the pemphigus variants, Hailey-Hailey, Darier's and Grover's diseases. In addition to these diseases, the dermatologist and dermatopathologist must be aware of entities that mimic classic acantholytic dermatoses and of rare disease variants, which are characterized by acantholysis. Read More

Br J Dermatol 2017 02;176(2):304-305

Kurume University Institute of Cutaneous Cell Biology, 67 Asahimachi, Kurume, Fukuoka, 830-0011, Japan.

Ann Dermatol Venereol 2017 Mar 24;144(3):169-175. Epub 2017 Feb 24.

Clinique dermatologique, hôpitaux universitaires de Strasbourg, 1, place de l'Hôpital, 67091 Strasbourg, France. Electronic address:

Background: Twenty-five years ago at the Journées Dermatologiques de Paris, Prof. Béatrice Crickx described a new association, "antimicrobial pustulosis and systemic lupus erythematosus", a condition now known as amicrobial pustulosis of the folds (APF). The aim of this study is to analyse the clinical and laboratory characteristics of APF and to outline the gradual advancement of knowledge regarding this disease. Read More

Medicine (Baltimore) 2017 Feb;96(8):e6184

Department of Hematology, The First Affiliated Hospital of Chongqing University of Medical Sciences, Chongqing, China.

Rationale: Paraneoplastic pemphigus (PNP) is an autoimmune syndrome associated with neoplasms. The treatment approach principally includes suppressing the immunity, but its therapeutic effect is not satisfying.

Patient Concerns: We report a case of paraneoplastic pemphigus linked to chronic lymphocytic leukemia in a 63-year-old man. Read More

Am J Dermatopathol 2017 03;39(3):230-231

Royal Liverpool and Broadgreen University Hospitals NHS Trust, Liverpool, United Kingdom.

Am J Dermatopathol 2017 Mar;39(3):197-198

Royal Liverpool and Broadgreen University Hospitals NHS Trust, Liverpool, United Kingdom.

J Am Acad Dermatol 2017 Jun 14;76(6):1109-1114.e2. Epub 2017 Feb 14.

Department of Specialized, Clinical, and Experimental Medicine, Division of Dermatology, University of Bologna, Bologna, Italy.

Background: Erosive pustular dermatosis of the scalp is a chronic eruption that leads to scarring alopecia.

Objective: The clinical, dermoscopic, and histopathological features and the course of the disease in 20 patients were reviewed and compared with the reports in the literature.

Methods: Gender, age at diagnosis, age at onset, duration, topography, predisposing factors, concomitant diseases, trichoscopy, histology, treatment, and outcome were taken into consideration. Read More

J Am Acad Dermatol 2017 Mar;76(3):560-562

Department of Dermatology, INSERM U1234, Rouen University Hospital, University of Rouen, France. Electronic address:

Am J Clin Dermatol 2017 Apr;18(2):287-297

Departments of Dermatology, Preventive Medicine and Medical Social Sciences, Northwestern University Feinberg School of Medicine, Suite 1600, 676 N. St. Clair St., Chicago, IL, 60611, USA.

Background: Little is known about the epidemiology of pediatric autoimmune blistering disorders (PAIBD).

Objective: We sought to determine the inpatient burden and comorbidities of PAIBD.

Methods: We analyzed data from the Nationwide Inpatient Sample from 2002 to 2012, which contained a representative 20% sample of all US hospitalizations. Read More

Cutis 2017 Jan;99(1):E23-E26

Broward Health Medical Center, Fort Lauderdale, Florida, USA.

Cutis 2017 Jan;99(1):E27-E30

Department of Dermatology, Rutgers-Robert Wood Johnson Medical School, Somerset, New Jersey, USA.

The reported sensitivity and specificity of enzyme-linked immunosorbent assay (ELISA) for bullous pemphigoid (BP) diagnosis is approximately 87% and 98%, respectively. These statistics suggest that ELISA is a reliable diagnostic test; therefore, the use of ELISA for BP diagnosis has increased. We report the case of a man who was diagnosed with BP and was treated for 3 years based on a positive ELISA for IgG against BP180. Read More

Cutis 2017 Jan;99(1):E1-E3

Department of Medicine, Faculty of Medicine, University Malaya, Kuala Lumpur, Malaysia.

A A Case Rep 2017 Feb;8(4):70-71

From the Departments of Pediatric Anesthesiology and Pediatric Plastic Surgery, Children's Memorial Hermann Hospital, McGovern Medical School, Houston, Texas.

A former 25-week-old neonate presented at 34 weeks postconceptual age with necrotizing fasciitis and purpura fulminans because of Group B Streptococcus infection. He was septic and coagulopathic when he was intubated, and the endotracheal tube was secured with adhesives. When he subsequently developed large purpuric, bullous lesions on the face and neck, he presented to the operating room for excision and debridement of his facial lesions. Read More

Aust Fam Physician 2017 Jan/Feb;46(1):45-47

Indian J Dermatol Venereol Leprol 2017 Mar-Apr;83(2):259-262

Department of Skin and VD, Indira Gandhi Government Medical College, Nagpur, Maharashtra, India.

Indian J Dermatol Venereol Leprol 2017 Mar-Apr;83(2):229-231

Department of Dermatology, Shenzhen Hospital, Southern Medical University, Shenzhen, Guangdong 518000, China.

Surg Pathol Clin 2017 Mar 28;10(1):57-88. Epub 2016 Dec 28.

Department of Oral and Maxillofacial Diagnostic Sciences, University of Florida, 1395 Center Drive, Gainesville, FL 32610, USA.

Immune-related disorders of the oral cavity may occur as primary disease process, secondary to systemic disease or neoplasm, or as a reaction to medications and other agents. The entities represented within this group may vary significantly by severity, clinical presentation, microscopic presentation, and special testing results. The selected immune-related conditions of the oral cavity in this article are categorized and presented by their prototypical tissue reaction patterns: vesiculobullous, including acantholytic and subepithelial separation; psoriasiform; spongiotic; and lichenoid reaction patterns. Read More

Health Qual Life Outcomes 2017 Feb 2;15(1):31. Epub 2017 Feb 2.

Institute of Dermatology and Department of Dermatology, The First Affiliated Hospital, Anhui Medical University, 81 Meishan Road, Hefei, Anhui, 230032, China.

Background: The autoimmune bullous diseases quality of life (ABQOL) questionnaire was recently developed by an Australian group and has been validated in Australian and North American patient cohorts. It is a 17-item, multidimensional, self-administered English questionnaire. The study aimed to validate the Chinese version of the ABQOL questionnaire and evaluate the reliability in Chinese patients. Read More

Arkh Patol 2016;78(6):9-16

State Research Center for Dermatovenereology and Cosmetology, Ministry of Health of Russia, Moscow, Russia.

Congenital epidermolysis bullosa (CEB) is an extensive group of hereditary skin diseases, the differential diagnosis of which is a challenge due to the rarity of this pathology and the diversity of its clinical manifestations. The determination of the type of CEB makes it possible to estimate its prognosis and to facilitate a prenatal diagnosis.

Aim: to optimize the morphological diagnosis of different types of CEB. Read More

BMJ Case Rep 2017 Jan 30;2017. Epub 2017 Jan 30.

Department of Dermatology, Wright State University Boonshoft School of Medicine, Dayton, Ohio, USA.

A man aged 56 years with a history of ulcerative colitis (UC) status postsubtotal colectomy was hospitalised with fevers, dry cough, eye redness and a new bloody, mucoid rectal discharge. 2 months prior to admission, the dermatologist had started him on dapsone for subcorneal pustular dermatosis but did not recognise that he had recently self-discontinued mesalamine enemas, inducing a flare of his UC. After a thorough inpatient evaluation, including flexible sigmoidoscopy, active UC involving the rectal stump was determined to be driving his dermatological and ophthalmological findings. Read More

J Nippon Med Sch 2016 ;83(6):216-222

Department of Dermatology, Nippon Medical School.

Toxic epidermal necrolysis (TEN) is a rare skin condition, most often drug-induced, known for its skin detachment and high mortality. In general, acute TEN is considered a T-cell mediated, type IV hypersensitivity disorder. It mostly results from a cumulative effect of risks from the drug structure, drug metabolism, HLA alleles and T cell clonotypes. Read More

Vestn Oftalmol 2016;132(6):11-19

Research Institute of Eye Diseases, 11 A, B Rossolimo St., Moscow, 119021, Russian Federation.

Cumulative biomicroscopic evidence is usually sufficient for the diagnosis of recurrent corneal erosion or bullous keratopathy, however, exploration of the disease pathogenesis requires subcellular-level visualization of corneal structure. In the current study, lanthanoid staining and scanning electron microscopy were employed to visualize quite a number of structures responsible for epithelium organization. In particular, the study proves possible the use of Ca/Nd isomorphous substitution at Ca2+ sites of cytoadherence proteins for visualization of corresponding cellular structures. Read More

J Invest Dermatol 2017 May 17;137(5):1104-1113. Epub 2017 Jan 17.

Department of Dermatology, Allergy, and Venereology, University of Lübeck, 23538 Lübeck, Germany. Electronic address:

Recruitment of neutrophils and eosinophils into the skin is a hallmark of pemphigoid diseases. The molecular cues regulating granulocyte recruitment into the skin and the individual contributions of neutrophils and eosinophils to pemphigoid diseases are, however, poorly understood. The lipid mediator leukotriene B4 (LTB4) is a potent granulocyte chemoattractant and is abundant in the skin blister fluid of bullous pemphigoid (BP) patients, but its pathogenic significance is unknown. Read More

Semin Diagn Pathol 2017 May 14;34(3):250-260. Epub 2016 Dec 14.

Section of Dermatopathology, Division of Surgical Pathology & Cytopathology, University of Virginia Medical Center, Charlottesville, VA, United States. Electronic address:

Several dermatoses are typified by the formation of spaces (blisters; bullae) within or beneath the epidermis. These may be acellular or filled with particular species of inflammatory cells. Etiological categories include infectious, immune-mediated, genetic, drug-related, and idiopathic lesions. Read More

Eye (Lond) 2017 Feb 20;31(2):301-332. Epub 2017 Jan 20.

Ocular Biology and Therapeutics, UCL Institute of Ophthalmology, London, UK.

This review is in two sections. The first section summarises 35 conditions, both common and infrequent, causing cicatrising conjunctivitis. Guidelines for making a diagnosis are given together with the use of diagnostic tests, including direct and indirect immunofluorescence, and their interpretation. Read More

J Cutan Pathol 2017 May 16;44(5):486-489. Epub 2017 Feb 16.

Department of Dermatology, University of Florida College of Medicine, Gainesville, Florida.

A total of 3 cases of pseudoherpetic transient acantholytic dermatosis (Grover disease) are presented, followed by a brief review of prior reports. All 3 patients were above the age of 60 and presented with a pruritic eruption composed of papules with or without vesicles distributed on the trunk. For all 3 patients, the clinical differential diagnosis included drug eruption but did not include Grover disease; in 1 patient, the clinical impression included herpesvirus infection. Read More

J Hum Genet 2017 Apr 19;62(4):485-489. Epub 2017 Jan 19.

Department of Human Genetics, Graduate School of Medicine, University of Tokyo, Tokyo, Japan.

A genome-wide association study (GWAS) for cold medicine-related Stevens-Johnson syndrome (CM-SJS) with severe ocular complications (SOC) was performed in a Japanese population. A recently developed ethnicity-specific array with genome-wide imputation that was based on the whole-genome sequences of 1070 unrelated Japanese individuals was used. Validation analysis with additional samples from Japanese individuals and replication analysis using samples from Korean individuals identified two new susceptibility loci on chromosomes 15 and 16. Read More