Nicole F. Velez

Dermatomyositis Induced by Anti–Tumor Necrosis Factor in a Patient With Juvenile Idiopathic Arthritis

IMPORTANCE Biologic therapies, including anti-tumor necrosis factor (TNF) agents, are increasingly used to treat a variety of autoimmune diseases. Paradoxically, these agents have been reported to induce some of the very diseases they were designed to treat, including dermatomyositis (DM). We describe the first case of anti-TNF-associated DM without muscle involvement presenting in an adult patient with a history of arthritis since childhood. This cutaneous eruption recurred after rechallenge with an alternate anti-TNF agent. OBSERVATIONS A 46-year-old man with juvenile idiopathic arthritis developed a pruritic cutaneous eruption while receiving etanercept. Given concern about a drug-induced eruption, etanercept therapy was discontinued and the cutaneous findings improved. However, after rechallenge with adalimumab, he developed similar findings consistent with the skin manifestations of DM. After discontinuation of all anti-TNF drug therapy and the addition of methotrexate sodium, his eruption improved. CONCLUSIONS AND RELEVANCE Because the use of these agents is increasing, practitioners should be aware of the possibility of anti-TNF-induced autoimmune disorders, including DM. The case described herein is unique in that anti-TNF-induced autoimmune disease occurred in a patient with existing arthritis since childhood and recurred with rechallenge, adding further evidence to support the existence of anti-TNF-induced DM.

Psoriasiform reactions to anti-tumor necrosis factor α therapy.

ObjectiveGiven increasing concern about the adverse effects of anti–tumor necrosis factor &agr; (anti–TNF-&agr;) medications, we sought to characterize psoriasiform eruptions in patients on these medications. MethodsIn a retrospective review of patients at the Brigham and Women’s Hospital combined dermatology-rheumatology clinic, we identified 13 patients (1 male and 12 female patients) who developed psoriasiform eruptions while on anti–TNF-&agr; medications. ResultsInciting medications were adalimumab, etanercept, and infliximab. Patients were on their inciting medication for a median time of 24 months and a mean time of 31.3 months before developing eruptions. Five of 7 patients experienced complete resolution of lesions with topical corticosteroids and discontinuation of anti–TNF-&agr; medications with the remaining 2 patients having partial improvement. One of the other 6 patients experienced complete resolution with topical corticosteroid treatment only, with the remaining 5 patients experiencing partial improvement. After changing anti–TNF-&agr; agents, 1 patient had partial improvement of psoriasiform lesions, and 7 patients had no improvement. ConclusionsAll of the main anti–TNF-&agr; medications currently used are capable of causing psoriasiform eruptions. Poor responders to topical agents, such as corticosteroids, may benefit from supplemental therapy aimed at the psoriasiform eruption or changing to a different class of immunomodulatory agents. Switching anti–TNF-&agr; medications had a low likelihood of improving psoriasiform skin reactions, further suggesting that these eruptions are a drug class effect.

Association of Advanced Leukemic Stage and Skin Cancer Tumor Stage With Poor Skin Cancer Outcomes in Patients With Chronic Lymphocytic Leukemia

IMPORTANCE Although it has been well established that patients with chronic lymphocytic leukemia (CLL) have an increased risk of developing skin cancer, few studies have investigated the effect of CLL stage on the risk of poor skin cancer outcomes. The present study of CLL staging assesses outcomes of melanoma, squamous cell carcinoma, and Merkel cell carcinoma in this high-risk population. OBJECTIVE To determine if progression of CLL measured by advanced Rai stage (III or IV) is associated with worse skin cancer outcomes. DESIGN, SETTING, AND PARTICIPANTS Twenty-year retrospective study at 2 academic centers in Boston, Massachusetts, of adults with CLL and either melanoma, squamous cell carcinoma, or Merkel cell carcinoma. MAIN OUTCOMES AND MEASURES Hazard ratios (HRs) for the development of poor skin cancer outcomes (local recurrence, nodal metastasis, distant metastasis, or death from skin cancer). RESULTS In total, 133 patients with 377 primary skin cancers and a median follow-up of 41 months were included. Squamous cell carcinoma predominated (92.0%). The risk of death from skin cancer was equivalent to the risk of death from CLL (13.5%). On multivariate analysis, advanced Rai stage (III or IV) at the time of the first skin cancer diagnosis (HR, 4.5; 95% CI, 2.3-8.9) and a high skin cancer tumor (T) stage (HR, 4.9; 95% CI, 2.2-10.8) were associated with poor skin cancer outcomes. Those with both a low skin cancer T stage and a low Rai stage (n = 265) had a low risk (5.3%; 95% CI, 3.2%-8.7%) of poor skin cancer outcomes. Those with a low T stage and a high Rai stage (n = 89) had a significantly higher risk of poor skin cancer outcomes (16.9%; 95% CI, 10.9%-26.0%). The 23 patients with a high T stage had high risks of poor outcomes regardless of CLL status (27.3% if a low Rai stage and 50.0% if a high Rai stage, with wide 95% CIs). CONCLUSIONS AND RELEVANCE In patients with CLL and non-basal cell carcinoma skin cancer, mortality is as high from skin cancer as from CLL. The Rai stage and skin cancer T stage should be considered when risk-stratifying patients with skin cancer. Regular communication between dermatologists and oncologists will help facilitate the identification of patients with CLL who are at high risk of having poor skin cancer outcomes.

Nail Surgery: Best Way to Obtain Effective Anesthesia

Nail procedures require an effective and reliable approach to anesthesia of the distal digit. Several techniques have been described in the literature. Herein, the relevant anatomy of the nail unit, pain pathways, anesthetic options, and several injection approaches to achieve complete anesthesia are reviewed. Also considered are the potential pitfalls and complications and their management. Ultimately, the physicians approach must be individualized to the patient, procedure, and setting.

Diffuse Dystrophic Calcinosis Cutis of the Scalp in a Patient With Scalp Discoid Lupus Erythematosus and Systemic Lupus Erythematosus

Author Affiliations: San Antonio Uniformed Services Health Education Consortium, Fort Sam Houston, Texas (Dr Elam); Dermatology Services, David Grant Medical Center, Travis Air Force Base, California (Dr McCleskey). Correspondence: Capt Elam, San Antonio Uniformed Services Health, Education Consortium, 3851 Roger Brooke Dr, No. 3600, Fort Sam Houston, TX 78234 (aelam6 @gmail.com). Conflict of Interest Disclosures: None reported. Disclaimer: The views expressed in this material are those of the authors and do not reflect the official policy or position of the US government, the Department of Defense, or the Department of the Air Force.

Panniculitis Associated With Dermatomyositis and Recurrent Ovarian Cancer

BACKGROUND Panniculitis is a rare cutaneous manifestation of dermatomyositis (DM), typically presenting as tender, erythematous subcutaneous nodules. Complications, such as pain, calcinosis, and lipoatrophy, are associated with high morbidity. While it has been suggested that panniculitis in DM may correlate with a better prognosis, our understanding of its true significance, prognostic implications, and management is limited by the paucity of reported cases. We describe the first reported case to our knowledge of DM-associated panniculitis in the setting of ovarian adenocarcinoma as well as in association with a recurrent malignancy. OBSERVATIONS A 63-year-old woman with a history of DM and recurrent ovarian adenocarcinoma presented with multiple painful, erythematous subcutaneous nodules on the bilateral upper arms, thighs, and buttocks. Histologic examination showed lymphoplasmacytic panniculitis with associated dermal mucin deposition, consistent with lobular panniculitis in association with connective-tissue disease. Treatment with oral methotrexate resulted in sustained clinical improvement over a 10-month period. CONCLUSIONS Although panniculitis in DM has previously been suggested to be a good prognostic indicator, our case report describes an association with ovarian adenocarcinoma and a recurrent malignancy. Methotrexate may be an effective treatment for panniculitis in DM.

Paraproteinemia-Associated Scleredema Treated Successfully With Intravenous Immunoglobulin

Discussion | There have been a variety of treatment techniques used for CRDD, including cryotherapy, surgical excision, irradiation, oral corticosteroids, dapsone, thalidomide, and isotretinoin. To our knowledge, the use of methotrexate alone or in combination with other agents has been reported in 9 cases of systemic Rosai-Dorfman, and a complete to partial response was reported in most cases.4 By contrast, methotrexate therapy has been reported in only 3 cases of CRDD, and partial or no improvement was reported.3,5,6 However, in all of these cases, the eruption had already been present for well over a year. In our patient, a lack of response to prednisone, preexisting diabetes, and significant disease burden prompted the choice of low-dose methotrexate, to which a complete clinical response was seen over 11 months. Though the exact cause of CRDD remains unknown, the presence of Epstein-Barr virus, human herpesvirus 6 by polymerase chain reaction, and reported eruption after vaccination7 with spontaneous remission over months to years suggests that CRDD is a benign reactive process involving a particular pattern of immune dysregulation. Early diagnosis remains a challenge in CRDD owing to its nonspecific clinical manifestations, including variable numbers of papules, nodules, plaques, or tumors. Timely diagnosis and initiation of methotrexate therapy may be key to effecting a rapid clinical remission while this disease remains in its active phase.

Nailing it: promoting nail procedural training in residency and beyond.

the nail avulsion, including sterilization, anesthesia, tourniquet placement, partial and total nail avulsion, and chemical or surgical matricectomy. Nail fold and bed skin are simulated by the rubber fingertip, which also serves to cover andprotect the plastic handmodel. Proximal nail fold reflection can be performed to expose the matrix. Nail structures and nail unit pathology can be illustrated using nail polish. Nail polish “cuticles” are a good reminder that these need to be released from the nail plate in live situations, as the plastic nail tip cannot be released until the nail polish is pushed off with a nail elevator.

Multiple Tense Bullae Localized to the Right Breast in a Woman in Her Seventies

Awoman in her 70s presented with a 1-month history of multiple tense bullae on the right breast associated with worsening erythema and tenderness. Four months prior, she had completed radiation therapy (RT) to this site (total energy dose, 50 Gy [to convert to rad,multiplyby 100]) for treatmentof invasiveductal breast carcinoma.Shewas initially treatedwithdicloxacillin sodiumforpossible bullous impetigo after a culture demonstrated Staphylococcus aureus but was then hospitalized for administration of broadspectrum intravenous antibiotics after demonstrating no clinical improvement. On physical examination, thereweremultiple tense 0.5to 3-cm bullae localized to the right breast (Figure, A and B). The bullae, aswell asmultiple erosions and surrounding erythema, occurredwithin thepreviously irradiated site, as demarcatedby radiation tattoos (Figure, A). There was no involvement of mucosal membranes. Punch biopsies were performed (Figure, C and D). What is your diagnosis?

Toxic Exanthems in the Adult Population

Frequently the internist is confronted with the nonspecific exanthematous eruption. While often a sign of a benign and self-limiting process, an exanthem also might herald the development of a more severe systemic syndrome. Infections, immune-mediated processes, drug reactions, a neoplasm, and familial syndromes with poor prognoses might all manifest initially with an exanthem. A thorough history and complete physical examination should be performed on all patients who present with an exanthem. Characterization of the exanthem morphology, other physical examination findings, and review of systems can help guide laboratory testing and ensure prompt diagnosis and early treatment of potentially life-threatening conditions. This article provides a brief overview of the conditions that must be considered in the evaluation of an ill patient with an exanthem.