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.

Darier disease, radiation therapy, and herpesvirus -- an unfortunate triad.

Darier disease (DD) is a rare autosomal dominant keratinizing disorder often characterized by brown scaly pruritic papules over the face, neck, and trunk. Herein is reported a patient who developed secondary cutaneous herpes simplex virus (HSV) following exacerbation of his DD as a result of radiation therapy. In November 2020, a 78-year-old man presented to clinic for a pruritic rash on his back consistent with DD. He had developed the rash after the conclusion of chemoradiation therapy for recently diagnosed urothelial carcinoma of the bladder with squamous differentiation. However, he returned two weeks later complaining of a marked worsening of the rash associated with a pain and burning sensations. Histopathology was non-conclusive, but the lesions were found to be positive for HSV-1 by PCR. The patient recovered without complication over a period of two weeks following a course of valacyclovir. There is precedent in the literature for ionizing radiation inducing flares of DD lesions in overlying skin. In addition, DD has been shown to put a patient at increased risk for secondary infections such as HSV. This case report demonstrates that HSV could pose a significant risk to those with DD receiving radiation therapy and thus could warrant prophylactic treatment.

Squamous cell carcinoma of the dorsal hands and feet after repeated exposure to ultraviolet nail lamps.

Gel nails are a common artificial nail option. Ultraviolet (UV) nail lamps are commonly used to cure gel nails. Ultraviolet A radiation is a known mutagen that penetrates into the nail bed. Although previously reported, the role of UV nail lamps in the carcinogenesis of both keratinocyte carcinoma and melanoma remains controversial. Herein, we report a patient taking the photosensitizing agent hydrochlorothiazide who developed numerous squamous cell carcinomas on the dorsal hands and feet with a 10-year history of UV nail light exposure every 2-3 weeks.

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.