A crack in the armor: Wolf isotopic response manifesting as cutaneous lupus.

Wolf isotopic response represents the development of skin lesions of one particular morphology occurring at the same site as another morphologically distinct and unrelated skin lesion. Cutaneous lupus erythematosus (CLE) is an autoimmune connective tissue disorder encompassing a wide range of phenotypes that may be associated with systemic involvement. Although CLE is a well-described entity with a broad spectrum, the occurrence of lesions manifesting as an isotopic response is rare. We present a patient with systemic lupus erythematosus who developed CLE in a dermatomal distribution following herpes zoster. When CLE lesions present in a dermatomal distribution, these cases may be difficult to distinguish from recurrent herpes zoster infection in an immunosuppressed patient. Therefore, they pose a diagnostic challenge and require balancing antiviral therapy with immunosuppression to sufficiently maintain adequate control of the autoimmune disease while addressing possible infections. To avoid treatment delay, clinicians should have elevated suspicion for an isotopic response when disparate lesions erupt in areas previously affected by herpes zoster or in cases of persistent eruptions at sites of prior herpes zoster. We discuss this case within the context of Wolf isotopic response and review the literature for similar cases.

Characterizing the adverse dermatologic effects of hydroxychloroquine: A systematic review.

BACKGROUND: Hydroxychloroquine is associated with myriad adverse dermatologic effects, most of which are poorly characterized by the literature, with unknown frequencies and risk factors. OBJECTIVE: To conduct a systematic review of the adverse dermatologic effects and predisposing factors of hydroxychloroquine toxicity. RESULTS: The review included 94 articles comprising 689 dermatologic adverse effects. A total of 21 unique dermatologic reactions were reported, most commonly drug eruption or rash (358 cases), cutaneous hyperpigmentation (116), pruritus (62), acute generalized exanthematous pustulosis (27), Stevens-Johnson syndrome or toxic epidermal necrolysis (26), hair loss (12), and stomatitis (11). Almost all underlying conditions were rheumatologic or autoimmune in nature, composed primarily of lupus erythematous (72% of all cases) and rheumatoid arthritis (14%). The range of reported mean cumulative dosages was wide, with some adverse reactions found after as little as 3 g or as much as 2500 g. LIMITATIONS: Dermatologic adverse events and primary diagnoses related to the use of hydroxychloroquine may be under-reported as only case reports and clinical trials that reported at least 1 dermatologic adverse effect were included. CONCLUSION: Although hydroxychloroquine is generally well tolerated, dermatologic adverse effects involving the skin, hair, or nails are a frequent and significant complication. Most of these reactions occurred after treatment of autoimmune conditions, often manifesting on the skin after a wide range of cumulative dosages.

Pro- and anti-inflammatory eicosanoids in psoriatic arthritis.

INTRODUCTION: Eicosanoids are biological lipids that serve as both activators and suppressors of inflammation. Eicosanoid pathways are implicated in synovitis and joint destruction in inflammatory arthritis, yet they might also have a protective function, underscoring the need for a comprehensive understanding of how eicosanoid pathways might be imbalanced. Until recently, sensitive and scalable methods for detecting and quantifying a high number of eicosanoids have not been available. OBJECTIVE: Here, we intend to describe a detailed eicosanoid profiling in patients with psoriatic arthritis (PsA) and evaluate correlations with parameters of disease activity. METHODS: Forty-one patients with PsA, all of whom satisfied the CASPAR classification criteria for PsA, were studied. Outcomes reflecting the activity of peripheral arthritis as well as skin psoriasis, Disease Activity Score (DAS)28, Clinical Disease Index (CDAI) and Body Surface Area (BSA) were assessed. Serum eicosanoids were determined by LC-MS, and the correlation between metabolite levels and disease scores was evaluated. RESULTS: Sixty-six eicosanoids were identified by reverse-phase LC/MS. Certain eicosanoids species including several pro-inflammatory eicosanoids such as PGE2, HXB3 or 6,15-dk,dh,PGF1a correlated with joint disease score. Several eicosapentaenoic acid (EPA)-derived eicosanoids, which associate with anti-inflammatory properties, such as 11-HEPE, 12-HEPE and 15-HEPE, correlated with DAS28 (Disease Activity Score) and CDAI (Clinical Disease Activity Index) as well. Of interest, resolvin D1, a DHA-derived anti-inflammatory eicosanoid, was down-regulated in patients with high disease activity. CONCLUSION: Both pro- and anti-inflammatory eicosanoids were associated with joint disease score, potentially representing pathways of harm as well as benefit. Further studies are needed to determine whether these eicosanoid species might also play a role in the pathogenesis of joint inflammation in PsA.

Non-uremic calciphylaxis in a patient with multiple rheumatologic diseases.

Non-uremic calciphylaxis is a rare, life-threatening condition characterized clinically by cutaneous necrosis and histologically by calcium deposition in small vessel walls. The etiology of non-uremic calciphylaxis remains the subject of ongoing speculation and debate. Herein we present a patient with calciphylaxis who had normal kidney function and numerous rheumatologic diseases, namely systemic lupus erythematosus (SLE), Sjogren syndrome (SS), and myasthenia gravis (MG). We review the pathophysiology, possible mechanisms, and management for non-uremic calciphylaxis.

Granulomatous fasciitis followed by morphea profunda: Is granulomatous fasciitis part of a spectrum of deep morphea? A case report and review of the literature.

Although eosinophilic fasciitis is known to be part of the deep morphea spectrum, this first report of the coexistence of granulomatous fasciitis and morphea profunda suggests that granulomatous fasciitis may also be a part of the spectrum of deep morphea.

Less common rheumatologic disorders: Current concepts of skin and systemic manifestations.

The cutaneous manifestations of the common rheumatologic disorders, such as systemic lupus erythematosus, dermatomyositis, and systemic sclerosis, are well known. In contrast, the dermatologic findings of less common rheumatologic disorders, including Sjögren syndrome, mixed connective tissue disease, and relapsing polychondritis, are less widely known. The cutaneous manifestations of these connective tissue disorders are reviewed.

Rituximab in the management of juvenile pemphigus foliaceus.

BackgroundPemphigus foliaceus (PF) is a blistering disorder most commonly presenting in middle age. As PF is restricted to the superficial epidermis, it is considered more benign than other pemphigus diseases. However, progression to severe disease is not uncommon. Although rituximab's efficacy has been well-documented in adults with refractory PF, little data is available on its role in adolescents.PurposeWe describe a patient with juvenile PF treated with rituximab and review the literature for similar cases.MethodsPubMed was searched for the terms: antibody, B cells, blistering, CD20, foliaceus, juvenile, pemphigus, rituximab, immunosuppression. As the first reported case of rituximab treated pemphigus was in 2001, only cases from 2001 and after were included. Juvenile PF was defined as disease diagnosis between ages 12-17.ResultsFive cases have been reported. The indication for rituximab in most cases was refractory PF unresponsive to systemic glucocorticoids and non-steroidal adjuvant therapies. All cases demonstrated significant improvement or complete remission and most experienced no adverse events.ConclusionsRituximab appears to be both well tolerated and efficacious for refractory juvenile PF. Therefore, it may be considered for severe cases of PF to avoid side effects associated with conventional glucocorticoid therapy.

Continuity of care in dermatology residency programs in the United States.

PURPOSE: As established by the AccreditationCouncil for Graduate Medical Education (ACGME),dermatology residents in the United States must participate in continuity clinic. This requirement may be achieved through multiple means, allowing for program variation. To better assess continuity clinic's role in resident learning, more data on this component of graduate medical education is needed. METHODS: An anonymous online survey was distributed via the American Board of Dermatology list serv to all U.S. dermatology residents. Continuity clinic organization, setting, frequency, and patient and preceptor characteristics were assessed; resident satisfaction and learning were compared. RESULTS: Of 231 responses, 7.8% reported continuity clinic daily, 77.1% weekly, 9.1% every other week, 3.0%monthly, 0.4% once every several months, and 2.2%only during certain blocks. Of the clinics reported,80.1% were "resident-run with attending" and 11.3%were attending-run. The rest were "resident-run with no attending" (0.9%), both resident and attending run(3.0%), or "other" (4.8%). Trainees in resident-run clinics (with attendings) reported greater continuity of care than those in attending-run clinics (p<0.001).Residents reported better teaching with attending presence during patient encounters than when attendings were present only if concerns were raised(p<0.01).

Discoid Lupus and Human Immunodeficiency Virus: A Retrospective Chart Review to Determine the Prevalence and Progression of Co-occurrence of these Conditions at a Single Academic Center.

CONTEXT: Discoid lupus erythematosus (DLE) and human immunodeficiency virus (HIV) are both disorders of the immune system. The pathophysiology of these diseases varies greatly as DLE is characterized by an overactive immune system that attacks normal host cells, whereas HIV is characterized by an exogenous attack on the immune system that depletes it of key cell types. Although the reason is unknown, co-occurrence of DLE and HIV is rare. AIMS: The goal of this study is to determine the prevalence of co-occurrence of DLE and HIV and to determine whether patients with both DLE and HIV share any clinical feature. SUBJECTS AND METHODS: The medical records of all patients seen within a single academic health center over a 20-year period were reviewed to determine the prevalence of cutaneous lupus, HIV, and co-occurrence of these conditions. The charts of patients diagnosed with both conditions were further reviewed to determine similarities between them. RESULTS: Of the 10,719 patients diagnosed with HIV and 182 patients diagnosed with cutaneous lupus, only 2 patients were diagnosed with both conditions. Both of these patients were diagnosed with DLE several years after being diagnosed with HIV. They had an undetectable HIV viral load, normal CD4 T-cell counts, and were on antiretroviral therapy when diagnosed with DLE. CONCLUSION: These results confirm that co-occurrence of DLE and HIV is rare. Although our study population was small, findings from these patients suggest that in HIV-positive patients, DLE manifestations occur when their HIV disease activity is minimal.

Characteristics of research tracks in dermatology residency programs: a national survey.

Pursuing research is encouraged in dermatology residency programs. Some programs offer specific research or investigative tracks. Currently, there is little data on the structure or scope of research tracks in dermatology residency programs. An anonymous online survey was distributed to the Association of Professors of Dermatology listserve in 2016. Program directors of dermatology residency programs in the United States were asked to participate and 38 of the 95 program directors responded. The survey results confirmed that a 2+2 research track, which is two years of clinical training followed by two years of research, was the most common investigator trackmodel and may promote an academic career at the resident's home institution. Further studies will help determine the most effective research track models to promote long-term outcomes.

Penicillamine-associated cutis laxa and milia en plaque - case report and review of cutaneous changes associated with penicillamine.

Penicillamine-induced skin changes are rare and include: hypersensitivity reactions, autoimmune reactions, and cutaneous elastoses. We report a case of a 73-year-old man with cystinuria taking penicillamine for over 50 years who presented with penicillamine-induced cutis laxa and milia en plaque. A brief review of penicillamine induced skin changes, specifically cutis laxa and milia en plaque, is presented.

Nodular Scleroderma Revisited: Systemic Sclerosis Presenting as Annular Keloidal Sclerotic Plaques.

BACKGROUND: Nodular scleroderma, also known as keloidal scleroderma, is a rare variant of systemic sclerosis. PURPOSE: The clinical features, pathologic findings and postulated pathogenesis of nodular scleroderma are discussed. METHODS: A woman with previously undiagnosed systemic sclerosis who presented with nodular scleroderma is described. Using the PubMed database, a literature search was performed on keloidal scleroderma, nodular scleroderma, and systemic sclerosis. RESULTS: Nodular scleroderma is characterized by firm plaques or nodules, which can mimic a keloid, that are typically located on the anterior orposterior upper trunk and the arms; they show pathologic changes of scleroderma, keloid, or hypertrophic scar. Akeloidal response of inflamed skin that is involved in an active fibrotic process inherent to systemic sclerosis, in individuals who are genetically predisposed to keloid formation, is the hypothesized pathogenesis. CONCLUSION: Nodular scleroderma is rare. The authors' patient presented with diarrhea, dysphagia, fatigue, Raynaud's phenomenon, shortness of breath, and annular keloidal plaques of morphea whose biopsy showed features of hypertrophic scar; additional studies confirmed the diagnosis of the nodular scleroderma variant of systemic sclerosis. The possibility of systemic sclerosis should be entertained in patients who present with nodularor keloidal plaques that morphologically resemble morphea and have histologic findings of a scar or a keloid-especially if there are associated symptoms suggestive for systemic sclerosis.

Epidermal multinucleated giant cells are not always a histopathologic clue to a herpes virus infection: multinucleated epithelial giant cells in the epidermis of lesional skin biopsies from patients with acantholytic dermatoses can histologically mimic a herpes virus infection.

BACKGROUND: Multinucleated giant cells in the epidermis can either be epithelial or histiocytic. Epithelial multinucleated giant cells are most often associated with herpes virus infections. PURPOSE: To review the histologic differential diagnosis of conditions with epithelial and histiocytic multinucleated giant cells-since multinucleated giant cells in the epidermis are not always pathognomonic of a cutaneous herpes virus infection-and to summarize dermatoses in which herpes virus infection has been observed to coexist. METHODS: Two individuals with acantholytic dermatoses whose initial lesional skin biopsies showed multinucleated epithelial giant cells suggestive of a herpes virus infection are reported. Using the PubMed database, an extensive literature search was performed on multinucleated giant cell (and epidermis, epithelial, and histiocytic) and herpes virus infection. Relevant papers were reviewed to discover the skin conditions with either multinucleated giant cells in the epidermis or coincident cutaneous herpes virus infection. RESULTS: Initial skin biopsies from patients with either pemphigus vulgaris or transient acantholytic dermatosis mimicked herpes virus infection; however, laboratory studies and repeat biopsies established the correct diagnosis of their acantholytic dermatosis. Hence, epidermal multinucleated giant cells are not always a histopathologic clue to a herpes virus infection. Indeed, epithelial multinucleated giant cells in the epidermis can be observed not only in the presence of infection (herpes virus), but also acantholytic dermatoses and tumors (trichoepithelioma and pleomorphic basal cell carcinoma). Histiocytic multinucleated giant cells in the epidermis can be observed in patients with either giant cell lichenoid dermatitis or lichen nitidus of the palms. CONCLUSIONS: Epithelial and histiocytic multinucleated giant cell can occur in the epidermis. Keratinocyte-derived multinucleated giant cells are most commonly associated with herpes virus infection; yet, they can also be observed in patients with skin tumors or acantholytic dermatoses. Cutaneous herpes simplex virus infection can coexist in association with other conditions such as acantholytic dermatoses, benign skin tumors, bullous disorders, hematologic malignancies, inflammatory dermatoses, and physical therapies. However, when a herpes virus infection is suspected based upon the discovery of epithelial multinucleated giant cells in the epidermis, but either the clinic presentation or lack of response to viral therapy or absence of confirmatory laboratory studies does not support the diagnosis of a viral infection, the possibility of a primary acantholytic dermatosis should be considered and additional lesional skin biopsies performed. Also, because hematoxylin and eosin staining is not the golden standard for confirmation of autoimmune bullous dermatoses, skin biopsies for direct immunofluorescence should be performed when a primary bullous dermatosis is suspected since the histopathology observed on hematoxylin and eosin stained sections can be misleading.

Annular plaques: An unusual manifestation of graft-versus-host disease.

Chronic cutaneous graft-versus-host disease (GVHD) classically presents with lichenoid papules or sclerotic plaques. This case highlights an unusual clinical manifestation of chronic GVHD and demonstrates that the skin morphology of chronic GVHD and cutaneous lymphoma may be similar. We report for the first time a case of annular scleroderma-like graft-versus-host disease in a patient following allogeneic stem cell transplant for CD30+ anaplastic large cell lymphoma. Treatment of these skin lesions with ultraviolet A1 (UVA1) phototherapy resulted in significant improvement.