A confounding clinically aggressive case of necrotizing granulomatous and suppurative dermatitis.

Superficial granulomatous pyoderma gangrenosum is a rare, superficial, vegetating form of pyoderma gangrenosum that tends to occur as a single lesion, most commonly on the trunk. Herein, we report a clinically confounding case of disseminated superficial granulomatous pyoderma gangrenosum in a patient with a 5-year history of painful and chronic ulcerations of the bilateral upper extremities and face in a sun exposed distribution. This was a diagnostically challenging case due to the treatment-refractory nature of our patient's skin lesions and the atypical clinical and histologic presentations encountered. We review our clinical decision process and acknowledge other entities that were considered during the clinical course of this case. Additionally, we discuss the lack of responsiveness to various treatment options with eventual successful clearance of this patient's active skin disease with initiation of adalimumab.

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. The patient was diagnosed with linear DLE, mimicking en coup de sabre, within Blaschko lines. The pathogenesis for DLE in association with chronic granulomatous disease is ambiguous; however, X-linked lyonization is crucial for both conditions and may explain cooccurrence of disease states.

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. To date, this young woman's skin changes have proven refractory to oral hydroxychloroquine and ultraviolet A1 phototherapy. Several aspects of this case including the patient's family history of Raynaud disease, her nodular sclerodermatous skin lesions, and the presence of U1RNP autoantibodies raised concern about her management with respect to future risk of developing systemic sclerosis.

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. In this report we will refer to this subgroup of patients as having idiopathic chronic eczematous eruption of aging (CEEA). Idiopathic CEEA must be distinguished from other established eczematous dermatoses. Idiopathic CEEA patients often require long-term systemic immunosuppressive drugs to make living bearable. Elder-onset atopic dermatitis is the most difficult of the known dermatoses to distinguish from idiopathic CEEA. Because of their clinical similarities we questioned whether dupilumab (Dupixent®), the first FDA-approved biologic for atopic dermatitis, might be valuable in the management of idiopathic CEEA. We report the case of an elderly man with idiopathic CEEA of four-years' duration who had a complete clinical response to the initiation of treatment with dupilumab. This case is presented to stimulate more discussion and systematic study of a possible role for dupilumab in otherwise-refractory idiopathic CEEA patients. We also propose a set of diagnostic criteria for idiopathic CEEA.

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. Cases reported in the English language wereorganized by drug class and compared with theresults of our previous review. RESULTS: From 55 selected publications, 95 qualifiedreports of DI-SCLE were identified. With theexception of a population-based study from Sweden,all other reports of DI-SCLE appeared as case reportsor small case series. Cases associated with protonpump inhibitors relative to all other medicationswere increased by 34.1%. Reports associated withantihypertensive and antifungal medicationsdecreased by 28.9% and 22.4%, respectively duringthis timeframe. The majority of new reports wereassociated with drugs not previously described.Greater than 70% of reports since August, 2009 werefrom European countries. CONCLUSIONS: The number of drugs associated withDI-SCLE is increasing. However, a form of publicationbias has likely contributed to this shift in reporting.There is a need for additional large, populationbasedstudies in this area.

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). Because we found no studies directly showing these cuticular products are in fact "hemosiderin-containing," we conducted a direct staining experiment in vivo using Prussian blue in order to increase our confidence that these products are indeed hemosiderin-containing and that the terminology is accurate for further use. EVIDENCE REVIEW: In July-December 2014, the MeSH function in PubMed was used to identify approximately 165 articles relating to capillaroscopy. We reviewed these articles for mention of microhemorrhages and hemosiderin deposits. In addition, we used PubMed and Google Scholar searches for "hemosiderin + nail", "Prussian Blue + nail", and "hemosiderin deposit." We found no papers reporting the use of Prussian Blue directly on nailfolds of patients with SSc and DM in vivo. FINDINGS: In our literature review, "microhemorrhages" and "hemosiderin deposits" were often used synonymously, yet they are clearly distinct entities. We present a case in which the presence of these deposits supported a diagnosis of amyopathic DM. We used Prussian blue staining solution to visualize the cuticular (eponychial) hemosoderin-containing deposits (CEHD) - distal cuticular products that reflect previous proximal nailfold microhemorrhage events. CEHD can serve as an indicator of active autoimmune disease, particularly in SSc and DM. CONCLUSIONS AND RELEVANCE: CEHD are in fact hemosiderin-containing deposits that can reflect the active inflammatory phase of microvascular injury occuring in autoimmune disorders such as DM and SSc. CEHD can be visualized and documented at the bedside with tools commonly available to any dermatologist (portable dermatoscope and compact digital camera).

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.  Owing to rising insurance co-pays, even patients who have prescription medication insurance coverage are beginning to experience challenges in this area.  This document was created to help patients and their families consider various strategies and programs that exist in 2015 for reducing their out-of-pocket costs for their prescription medications.  We believe that this information can also be helpful to healthcare providers when counseling patients about managing rapidly rising prescription drug costs.  An effort has been made to make this document readable to patients and their families as well as to healthcare providers.

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). Patients presenting with ADM have been reported by investigators of multiple nationalities to be at risk for rapidly progressive, potentially fatal ILD (2-5). In addition, a new autoantibody, anti-CADM-140, has been reported to be a risk factor for the development of interstitial lung disease in CADM patients (6-9). It has been argued that ADM patients may be at increased risk of developing internal malignancy compared to the general population, though its rate in comparison to classic DM (CDM) needs further study (1, 10-12). In our population, 41% of CADM patients were previously classified as LE or UCTD. We conclude that ADM is a real entity that makes up a significant portion of the DM disease. It is important to formally recognise amyopathic DM as a subset of DM as it carries increased risk of ILD and possibly malignancy. Without appropriate disease classification, the opportunity for ILD and malignancy screening may be missed.

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.e., the Rudolph sign--as in Rudolph The Red Nosed Reindeer). This symptom complex responded rapidly to topical intranasal mupirocin ointment treatment after having previously failed other treatments including a topical intranasal triple antibiotic ointment and oral doxycycline. This case is instructive as it describes a heretofore under-recognized, but not uncommon, mucocutaneous clinical entity that has been linked to more serious head and neck infections and likely has relevance to the intranasal carriage of Staphylococcus aureus. We review the limited published literature on this mucocutaneous disorder including its nosology and propose future lines of investigation for better defining its clinical significance and pathogenesis.

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. The clinical value of rituximab in other immune-mediated blistering diseases has been less thoroughly examined. We report a case of a woman who presented initially with the Brunsting-Perry phenotype of cicatricial pemphigoid who subsequently developed severe generalized subepidermal blisters healing with scarring and milia formation thought to be clinically compatible with epidermolysis bullosa acquisita, although type VII collagen autoantibodies were never identified. Treatment with a number of conventional systemic agents was unsuccessful and complicated by methicillin-resistant Staphylococcus aureus-induced cutaneous ulcers and near-fatal gram-negative sepsis. This woman has enjoyed an 18-month complete clinical remission after a single inductive 4-week cycle of intravenous rituximab. This outcome supports the idea that systemic memory B-cell depletion with drugs such as rituximab should be considered for therapeutically refractory subepidermal autoimmune blistering diseases in addition to intraepidermal autoimmune blistering diseases. A potential role for the immunologic phenomenon of epitope spreading in the generation of overlapping features of autoimmune blistering diseases, and its contribution to therapeutic refractoriness ("hardening"), is discussed.

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. DESIGN: Retrospective chart review of 271 consecutive dermatologic consultations from primary ward teams between January 20, 1998, and May 19, 1999. SETTING: Non-dermatology in-patient services at a Midwestern state-supported university hospital system in the U.S. PATIENTS: Patients hospitalized on non-dermatology wards with skin problems prompting a formal dermatologic consultation. INTERVENTIONS: None. MAIN OUTCOME MEASURE: Prevalence of dermatologic conditions that are most frequently misdiagnosed on non-dermatology in-patient services. RESULTS: Seventy-six percent of the dermatologic consults were requested by Internal Medicine, Surgery, and Psychiatry departments. Common skin conditions accounted for a large majority of dermatologic consultations including: dermatitis (21.0%) and drug eruption (10.0%). The primary ward team submitted a correct dermatologic diagnosis in only 23.9 percent of cases. Dermatology consultation resulted in a change in or addition to treatment in 77 percent of patients. CONCLUSIONS: Our results suggest that common skin conditions account for a large majority of dermatologic consultations in a University hospital setting. Modern hospital ward teams continue to struggle with accurately recognizing and appropriately managing common skin problems resulting in inappropriate treatment, wasted resources, and prolonged hospitalization. Increasing medical student and house staff knowledge and experience in the diagnosis and management of common skin disorders could help address this problem.

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. Quinacrine is currently unavailable in the USA, which has taken away an important component of the treatment regimen of patients with CLE. This paper reviews the evidence of available local and systemic therapies in order to assist providers in choosing alternative treatments for patients who previously benefited from quinacrine therapy.

Cutaneous lupus erythematosus: molecular and cellular basis of clinical findings.

Cutaneous lupus erythematosus (CLE) is a heterogeneous autoimmune disease with welldefined skin lesions, including acute CLE, subacute CLE, chronic CLE and intermittent CLE. In the first part of the review, we discuss the variable relationships that exist between the different clinical forms of CLE and the risk of systemic disease activity. Furthermore, we focus on the annular and papulosquamous forms of subacute CLE and emphasize dermal scarring as a characteristic feature of chronic discoid skin disease in contrast to other subtypes of CLE. Various environmental factors influence the clinical expression of CLE and a striking relationship has emerged between sunlight exposure and the various subtypes of this disease. In the second part, we review the evidence for the abnormal long-lasting photoreactivity in CLE, with an overview of the molecular and cellular factors that may underlie this abnormality. In particular, we discuss the role of UV-mediated induction of apoptosis, mediators of inflammation, such as cytokines and chemokines, nitric oxide, T cell-mediated injury, and the influence of regulatory CD4+CD25+ T cells. However, a complete understanding of the diverse pathophysiologic mechanisms and interactions in CLE does not exist and, as there is yet no convincing animal model of CLE, many studies remain descriptive in nature.

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. Multisystem involvement as a component of systemic LE (SLE) is common, with prominent musculoskeletal symptoms. SCLE is highly photosensitive, with predominant distribution on the upper back, shoulders, neck, and anterior chest. SCLE is frequently associated with positive anti-Ro antibodies and may be induced by a variety of medications. Classic discoid LE is the most common form of CCLE, with indurated scaly plaques on the scalp, face, and ears, with characteristic scarring and pigmentary change. Less common forms of CCLE include hyperkeratotic LE, lupus tumidus, lupus profundus, and chilblain lupus. Common cutaneous disease associated with, but not specific for, LE includes vasculitis, livedo reticularis, alopecia, digital manifestations such as periungual telangiectasia and Raynaud phenomenon, photosensitivity, and bullous lesions. The clinical presentation of each of these forms, their diagnosis, and the inter-relationships between cutaneous LE and SLE are discussed. Common systemic findings in SLE are reviewed, as are diagnostic strategies, including histopathology, immunopathology, serology, and other laboratory findings. Treatments for cutaneous LE initially include preventive (e.g. photoprotective) strategies and topical therapies (corticosteroids and topical calcineurin inhibitors). For skin disease not controlled with these interventions, oral antimalarial agents (most commonly hydroxychloroquine) are often beneficial. Additional systemic therapies may be subdivided into conventional treatments (including corticosteroids, methotrexate, thalidomide, retinoids, dapsone, and azathioprine) and newer immunomodulatory therapies (including efalizumab, anti-tumor necrosis factor agents, intravenous immunoglobulin, and rituximab). We review evidence for the use of these medications in the treatment of cutaneous LE.

Sarcoidal alopecia as a mimic of discoid lupus erythematosus.

Cicatricial alopecia presents a diagnostic challenge to clinicians. In particular, lesions of cutaneous sarcoidosis of the scalp may resemble discoid lupus erythematosus (DLE). We report two cases of sarcoidal alopecia originally thought to represent DLE scarring plaques of the scalp. A subsequent histopathologic diagnosis of sarcoidal alopecia was made. These cases highlight the importance of making the correct diagnosis of sarcoidal alopecia versus DLE so that a patient can receive the correct work-up and definitive treatment. These cases illustrate the fact that there should be a low threshold for biopsy of cicatricial plaques on the scalp.

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. And, LE is clinically-expressed quite variably from one individual to another making prognosis difficult. As such it can be very challenging to know what the optimal treatment approach might be for new patients presenting with this potentially-fatal disorder. Dr. Gilliam's major career focus was to better understand the complex relationships that exist between the clinical expression of LE in the skin and vital internal organs. In the late 1970s, Dr. Gilliam first described a new clinical form of LE skin disease that he designated as "subacute cutaneous LE." Subacute cutaneous LE would subsequently serve as the linchpin for a new classification scheme for LE skin disease that would later become known as the "Gilliam classification" of LE skin disease. In addition, he was among the first to apply modern immunologic insight to the classification of cutaneous LE. This work was carried out in the Divisions of Dermatology and Rheumatology and the Department of Dermatology at the University of Texas Southwestern Medical School in Dallas, Texas (UT Southwestern) starting in 1972. Dr. Gilliam served as the Founding Chairman of the Department of Dermatology at UT Southwestern in 1982, 2 years before his untimely death. Dr. Gilliam's clinical research accomplishments were matched by his ability to identify and encourage like-minded young people. A high percentage of his trainees went on to successful academic research careers and leadership positions in American Dermatology. Dr. Gilliam's untimely death from cancer deprived several generations of dermatologists and rheumatologists the benefit of his warm support and insightful guidance. In addition, American Dermatology and Rheumatology leadership organizations were deprived of his strong leadership skills.

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. In addition, while HCQ is known to have clinical benefits for the cutaneous manifestations of DM, I would also like to explore the secondary hypothesis that HCQ might have steroid-sparing effects on one or more of the systemic manifestations of DM.

Pathophysiology of cutaneous lupus erythematosus.

Cutaneous lupus erythematosus (LE; syn LE-specific skin disease) is an autoimmune disease with well-defined skin manifestations often accentuated in a photodistribution and frequently associated with specific autoantibodies. These clinical observations have led to numerous laboratory studies related to the role of ultraviolet light, as well as studies of the cascade of immunologic events involved in the pathogenesis of cutaneous LE. We discuss the epidemiologic, clinical, and laboratory findings of cutaneous LE, including the classification of disease subsets. We review the evidence for abnormal photoreactivity in LE with an overview of the cellular, molecular, and genetic factors that may underlie this abnormality. As there is yet no convincing animal model of cutaneous LE, many studies remain descriptive in nature. To arrive at an understanding of the potential mechanisms underlying the development of cutaneous lupus, we discuss the role of ultraviolet light-mediated induction of apoptosis, antigen presentation, genetic factors, and mediators of inflammation. In addition, we consider the role and importance of humoral and cellular factors, synthesizing the current understanding of the pathophysiology of cutaneous lupus.

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., "clinically-amyopathic DM" (CADM)] and were at risk for acute, rapidly-progressive form of interstitial lung disease (ILD). As an academic dermatologist in the United States of America (USA) having a career-long interest in the CADM subset, I would like to share my perspective on the results of the work by Allenbach and colleagues and offer some suggestions for additional study in this area. But to do so most effectively, I first would like to review the clinical concept of CADM and its association with anti-MDA5 antibody production and a potentially-fatal form of (ILD).

A systematic review of adult-onset clinically amyopathic dermatomyositis (dermatomyositis siné myositis): a missing link within the spectrum of the idiopathic inflammatory myopathies.

OBJECTIVE: Classical dermatomyositis (CDM) patients display the hallmark cutaneous manifestations of dermatomyositis (DM), proximal muscle weakness, and laboratory evidence of myositis. The epidemiology and management of both adult-onset and juvenile-onset CDM has been well characterized. However, the clinical significance of the hallmark inflammatory cutaneous manifestations of DM occurring in individuals who have no clinically significant muscle weakness and normal muscle enzymes for prolonged periods of time (ie, 6 months or longer) has not been clear. The term amyopathic DM (ADM) (synonymous with DM siné myositis) has been proposed to draw attention to such individuals. A related form of DM, "hypomyopathic DM" [HDM], is the presence of DM skin disease for 6 months or longer in individuals who have no muscle weakness but who are found to have some evidence of muscle inflammation upon testing (muscle enzyme levels, electromyogram, muscle biopsy, muscle magnetic resonance imaging [MRI]). Clinically amyopathic DM (CADM) is a designation that has been proposed for patients having either ADM or HDM. The clinically amyopathic component of this designation was coined to emphasize the fact that the only clinical problem being experienced by these patients at the time of diagnosis is their DM skin disease. Our personal experience suggests that the CADM subphenotype might be more prevalent in adults than has been thought previously. To test this hypothesis and address questions relating to the optimal management and prognosis of such patients, we have systematically reviewed the published literature in this area. METHODS: We carried out a systematic review of the published literature on adult-onset CADM as defined in Table 1 through May 1, 2004. RESULTS: We identified 291 adult-onset CADM cases (18 years or older) reported from over 19 countries. The average duration of DM skin disease was 3.74 years (range, 6 months [by definition] to > 20 years), and 73% were female. Among 37 patients with HDM who were identified, the average duration of disease was 5.4 years, and none had developed clinically significant weakness at the time of the reports. Thirty-seven of the reported CADM patients developed muscle weakness greater than 6 months after onset of their skin disease (15 months to 6 years). For the sake of this discussion, such patients have been analyzed under the designation of "CADM --> CDM." Somewhat surprisingly, 36/291 (13%) of the identified published CADM patients developed interstitial lung disease. Incidental to our review, we also identified 10 published cases of individuals having DM skin disease and interstitial lung disease without muscle weakness, 7 of whom died from interstitial lung disease less than 6 months after onset of their DM skin disease (the term pre-myopathic DM coined by others has been used here to refer to such patients). In addition, an associated internal malignancy was found in 41/291 (14%) of the identified CADM cases. A positive antinuclear antibody was reported in 63% and myositis-specific autoantibodies (eg, Jo-1, Mi-2) in only 3.5% of the reported CADM patients in which such data were available. CONCLUSIONS: The results of this analysis suggests that the CADM subphenotype is more common than has been thought previously and that such patients may comprise a relatively high proportion of DM patients followed by dermatologists. Some CADM patients also have been observed to develop overt proximal muscle weakness years after onset of their DM skin disease. In addition, CADM patients appear to be at risk of developing the same potentially fatal disease associations/complications for which CDM patients are at risk (eg, interstitial lung disease and internal malignancy). Population-based studies of the epidemiology and optimal management of CADM patients, including efforts to identify risk factors associated with potentially fatal outcomes such as late-onset muscle weakness, interstitial lung disease, and malignancy, are needed. As an incidental finding to this literature review, we also identified a small number of reported cases of often-fatal interstitial lung disease occurring shortly after the onset of DM skin disease (< 6 months) in the complete absence of muscle weakness. This subphenotype, referred to as "pre-myopathic DM," is one with which dermatologists should be aware as early diagnosis and aggressive management can be lifesaving.