Linear discoid lupus erythematous simulating en coup de sabre morphea in a female chronic granulomatous disease carrier.

Discoid lupus erythematosus (DLE), a subtype of chronic cutaneous lupus may be observed in a linear pattern. A 21-year-old woman with history of chronic granulomatous disease state presented to our clinic for a chronic six-year skin eruption on her left eyebrow, left cheek, and left forehead. A punch biopsy of involved left forehead skin was performed and revealed perivascular and periadnexal lymphohistiocytic infiltrate without features of morphea or panniculitis, confirming the histopathologic changes of cutaneous lupus erythematous.

Questions raised by a case of adult-onset linear nodular scleroderma.

Morphea presenting clinically with nodular or keloidal skin changes is extremely rare. Nodular scleroderma or keloidal morphea presenting in a linear distribution is even more uncommon. We present an otherwise healthy young woman with unilateral, linear, nodular scleroderma and review the somewhat confounding earlier literature in this area.

Candidate drug replacements for quinacrine in cutaneous lupus erythematosus.

Cutaneous lupus erythematosus (CLE) is a disfiguring and potentially disabling disease that causes significant morbidity in patients. Antimalarials are an important class of medication used to treat this disease and have been the first-line systemic therapy since the 1950s. Quinacrine, in particular, is used as an adjuvant therapy to other antimalarials for improved control of CLE.

James Neil Gilliam, MD-the career arc of a patient-oriented translational clinical investigation changemaker in rheumatologic skin disease.

James Neil Gilliam, MD, was an American academic physician who was trained in internal medicine, dermatology, dermatopathology and rheumatology. This "quadruple-threat" profile of postgraduate medical training provided him with a rather unique perspective on genetically-complex, environmentally-impacted human autoimmune disorders such as lupus erythematosus (LE). Both the skin and vital internal organs can be damaged by LE autoimmunity.

Aminoquinoline antimalarial therapy in dermatomyositis-are we missing opportunities with respect to comorbidities and modulation of extracutaneous disease activity?

It is now widely accepted that long-term aminoquinoline antimalarial therapy with hydroxychloroquine (HCQ) can mitigate one of the most important comorbidities of systemic lupus erythematosus (LE)-atherosclerotic cardiovascular disease (ASCVD). Increasing evidence suggests that idiopathic inflammatory myopathy (IIM) patients have a risk for ASCVD comorbidity that is similar to that of systemic LE. I would like to explore the primary hypothesis that long-term HCQ therapy could provide those with IIM, especially dermatomyositis (DM) patients, an ASCVD comorbidity benefit similar to that of systemic LE.

A possible role for dupilumab (Dupixent) in the management of idiopathic chronic eczematous eruption of aging.

Aging individuals can develop generalized, exquisitely-pruritic, eczematous eruptions of uncertain etiology that can be therapeutically-refractory and life-altering. Limited information exists in the literature to guide clinicians in the diagnosis and management of such patients. It is suggested that in approximately 40% of such patients a known cause for their chronic pruritic eruptions cannot be identified.

A pilot study evaluating biomarker development for drug-induced chronic eczematous eruptions of aging individuals.

Background: Identifying the drug(s) responsible for drug-induced chronic eczematous eruptions of aging individuals (CEEA) is a clinical challenge in patients on multiple medications. Reliable testing methods and biomarkers are needed to identify the causative agent and allow simultaneous assessment of T-cell responses to multiple drugs being taken concurrently. This study examined the feasibility of using , drug-specific T cell activation responses as a biomarker for drug-induced CEEA.

MDA5 autoantibody-another indicator of clinical diversity in dermatomyositis.

Allenbach and colleagues have recently reported for the first time the results of an intriguing study of the histopathologic, immunopathologic and gene expression differences in muscle biopsy tissue from adult dermatomyositis (DM) patients who do and do not have circulating MDA5 autoantibodies (anti-MDA5). Anti-MDA5 were originally identified in a clinically-defined subset of DM patients whose disease was expressed predominately in the skin for unusually long periods of time without accompanying muscle weakness [i.e.

An update in drug-induced subacute cutaneous lupus erythematosus.

Background: It has been over three decades sincethe first report of drug-induced subacute cutaneouslupus erythematosus (DI-SCLE) was described. Withan increasing variety of implicated drugs and thepotential for publication bias, we must consider: 1) hasthere been a change in drugs most often reported inDI-SCLE over time, and, 2) if so, of which drugs shouldclinicians be most suspicious in the setting of possibleDI-SCLE?

Objective: To determine which drug(s) present thehighest risk for inducing DI-SCLE.

Methods: The PubMed database was queried forreports of DI-SCLE from August, 2009 until May,2016.

Proximal nailfold microhemorrhage events are manifested as distal cuticular (eponychial) hemosiderin-containing deposits (CEHD) (syn. Maricq sign) and can aid in the diagnosis of dermatomyositis and systemic sclerosis.

Importance: Many patients present with cutaneous signs and symptoms that suggest a diagnosis on the autoimmune disease spectrum. During the "acute phase" of disease activity, patients with systemic sclerosis (SSc) and dermatomyositis (DM) have characteristic nailfold findings, including dilated capillaries, microhemorrhages, and hemosiderin deposits.

Objective: To review the literature on the presentation of microhemorrhages and to highlight the differences (in terms of terminology, characterization, and clinical relevance) between proximal microhemorrhage events and the distal products, often thought of as "hemosiderin deposits" located in the cuticle (eponychium).

How to reduce out-of-pocket costs for prescription medications.

The cost of prescription medicines has recently been rising faster than other healthcare costs.  This is also true for traditionally inexpensive generic medications that have long served as a fundamental healthcare safety net in the USA.  These changes increasingly present challenges for individuals to obtain common medications.

Chronic eczematous eruptions in the aging: further support for an association with exposure to calcium channel blockers.

Importance: Dermatologists frequently encounter patients of advanced age presenting with chronic eczematous eruptions of uncertain etiology. When a drug-induced cutaneous eruption is suspected, identifying the responsible drug(s) is a complex clinical challenge.

Objective: To determine whether certain drug classes, and in particular calcium channel blockers, are associated with chronic eczematous eruptions in the aging (CEEA) in the United States.

The importance of including amyopathic dermatomyositis in the idiopathic inflammatory myositis spectrum.

The objective of this review is to summarise the published evidence that supports the existence of amyopathic dermatomyositis (ADM) and its clinical significance including risk for rapidly progressive, fatal interstitial lung disease (ILD) and possible risk for internal malignancy. By establishing such inherent risks, we hope to establish the importance of formally recognising ADM as a subset of dermatomyositis (DM). Population-based epidemiologic studies have suggested that amyopathic DM might account for 20% of the total population of dermatomyositis (DM) patients (1).

The Rudolph sign of nasal vestibular furunculosis: questions raised by this common but under-recognized nasal mucocutaneous disorder.

Nasal vestibular furunculosis is a mucocutaneous disorder commonly seen in the general population. Despite its prevalence in clinical practice, it has been inconsistently described and labeled in the medical literature. We present a case of nasal vestibular furunculosis presenting as recurrent exquisitely tender unilateral erythema and edema of the nasal tip (i.

Sustained clinical response to rituximab in a case of life-threatening overlap subepidermal autoimmune blistering disease.

The conventional treatment for the autoimmune bullous skin diseases is broad-spectrum immunosuppressive regimen typically combining systemic corticosteroids with adjuvant immunosuppressive therapeutic agents. Orphan diseases in the pemphigus, pemphigoid, and epidermolysis bullosa acquisita groups of clinical disorders are often clinically severe, requiring long-term treatment with such drugs or drug combinations. Rituximab, a chimeric recombinant monoclonal antibody targeting CD20(+) B cells, has recently been suggested to be effective in the treatment of pemphigus with relatively few adverse effects.

Epidemiology and outcomes of dermatology in-patient consultations in a Midwestern U.S. university hospital.

Context: A paucity of data exists concerning the utilization of in-patient dermatologic consultations. Previous studies on this subject have indicated a knowledge deficit of primary care providers with regard to common dermatoses, prompting a need for more effective teaching mechanisms in this area.

Objective: To identify dermatologic conditions in the in-patient setting that are frequently misdiagnosed by non-dermatologists in order to improve future patient care and cost reduction through physician education.

Juvenile-onset clinically amyopathic dermatomyositis: an overview of recent progress in diagnosis and management.

Juvenile-onset amyopathic dermatomyositis is an uncommon variant of juvenile-onset dermatomyositis (JDM), characterized by the hallmark cutaneous features of dermatomyositis for at least 6 months without clinical or laboratory evidence of muscle disease. Cutaneous calcinosis, vasculopathy, and interstitial lung disease frequently complicate the course of classic JDM (typical JDM with myositis) but are infrequent in amyopathic JDM. Recent literature suggests that approximately 75% of amyopathic JDM patients will remain free from muscle disease after years of follow-up, while approximately 25% of patients will evolve to having classic JDM.

Cutaneous lupus erythematosus: issues in diagnosis and treatment.

Cutaneous lupus erythematosus (LE) may present in a variety of clinical forms. Three recognized subtypes of cutaneous LE are acute cutaneous LE (ACLE), subacute cutaneous LE (SCLE), and chronic cutaneous LE (CCLE). ACLE may be localized (most often as a malar or 'butterfly' rash) or generalized.

Lichenoid tissue reaction/interface dermatitis: clinical and histological perspectives.

A number of uncommon, clinically diverse and poorly understood inflammatory skin diseases are linked by the presence of a set of histopathological elements that have traditionally been referred to as the "lichenoid tissue reaction/interface dermatitis" (LTR/IFD). The prototypic skin disease in this category is lichen planus. However, the LTR/IFD can also be seen in skin disorders associated with systemic illnesses (lupus erythematosus, dermatomyositis), and the skin changes of potentially fatal disorders such as graft-versus-host disease, Stevens-Johnson syndrome, and toxic epidermal necrolysis.