Pyoderma gangrenosum: a presenting sign of myelodysplastic syndrome in undiagnosed Fanconi anemia.

A 26-year-old man with a history of congenital bilateral microtia, unilateral renal agenesis, left aural atresia, and right external auditory canal occlusion admitted for right rib cartilage graft harvest and left ear re-construction. Following surgery, an ulceration with violaceous borders and a yellow fibrinous base unresponsive to broad-spectrum antibiotics developed at the harvest site. The wound was expanding and not responsive to systemic broad-spectrum antibiotics. Biopsy revealed a dense dermal infiltrate of neutrophils with negative tissue cultures consistent with pyoderma gangrenosum (PG). He was treated with systemic, intralesional, and topical steroids, as well as doxycycline. Three weeks after the diagnosis of PG, he was found to have persistent anemia and leukopenia. Bone marrow aspiration analysis was consistent with hypocellular myelodysplastic syndrome and genetic testing was consistent with Fanconi anemia. There is a well-known association of PG with hematological disorders. Fanconi anemia is a rare genetic hematologic disorder with congenital defects leading to bone marrow failure and malignancy in long-standing disease. In our patient, we consider his development of PG a paraneoplastic sign associated with the onset of his hypocellular myelodysplastic syndrome.

Medical Malpractice in Dermatology-Part II: What To Do Once You Have Been Served with a Lawsuit.

Facing a malpractice lawsuit can be a daunting and traumatic experience for healthcare practitioners, with most clinicians naïve to the legal landscape. It is crucial for physicians to know and understand the malpractice system and his or her role once challenged with litigation. We present part II of a two-part series addressing the most common medicolegal questions that cause a great deal of anxiety. Part I focused upon risk-management strategies and prevention of malpractice lawsuits, whereas part II provides helpful suggestions and guidance for the physician who has been served with a lawsuit complaint. Herein, we address the best approach concerning what to do and what not to do after receipt of a legal claim, during the deposition, and during the trial phases. We also discuss routine concerns that may arise during the development of the case, including the personal, financial, and career implications of a malpractice lawsuit and how these can be best managed. The defense strategies discussed in this paper are not a guide separate from legal representation to winning a lawsuit, but may help physicians prepare for and cope with a medical malpractice lawsuit. This article is written from a US perspective, and therefore not all of the statements made herein will be applicable in other countries. Within the USA, medical practitioners must be familiar with their own state and local laws and should consult with their own legal counsel to obtain advice about specific questions.

Medical Malpractice in Dermatology-Part I: Reducing the Risks of a Lawsuit.

Malpractice risk is a common source of concern for the practicing physician. Dermatologists experience fewer lawsuits than most other specialists in medicine, but the risk is not negligible. All physicians should familiarize themselves with areas of potential risk and avoid medico-legal pitfalls. We present Part I of a two-part series addressing medico-legal questions common to most practitioners that cause a great deal of anxiety. Part I will focus upon risk management and prevention of future malpractice lawsuits, and Part II deals with suggestions and guidance once a lawsuit occurs. Herein, we discuss the primary sources of malpractice lawsuits delivered against healthcare practitioners including issues with informed consent, patient noncompliance, medical negligence, and inappropriate documentation, including use of electronic medical records. The overall goal is to effectively avoid these common sources of litigation. The risk management strategies discussed in this paper are relevant to the everyday practitioner and may offer physicians some degree of protection from potential liability.

Practice Gaps: Drug Reactions.

The term "drug reactions" is relevant to dermatology in three categories of reactions: cutaneous drug reactions without systemic features, cutaneous drug reactions with systemic features, and systemic drugs prescribed by the dermatologist with systematic adverse effects. This article uses examples from each of these categories to illustrate several important principles central to drug reaction diagnosis and management. The information presented will help clinicians attain the highest possible level of certainty before making clinical decisions.

Multiple keratoacanthomas occurring in surgical margins and de novo treated with intralesional methotrexate.

Keratoacanthomas (KAs) are common skin lesions known for their rapid growth and spontaneous regression. Keratoacanthomas also can occur in sites of prior trauma, such as surgical scars. We report a case of multiple KAs occurring in the site of trauma from a prior surgery and de novo as well as the response to treatment with intralesional methotrexate (MTX).

Important controversies associated with isotretinoin therapy for acne.

Isotretinoin is a remarkably effective drug for severe, recalcitrant acne vulgaris. Soon after the drug's release in the early 1980s, a number of important adverse effects were reported subsequently leading to a variety of medical and medicolegal controversies. Three of these controversies will be highlighted concerning the putative role of isotretinoin in (1) depression and suicide, (2) inflammatory bowel disease, and (3) iPledge and pregnancy prevention programs. It appears that a very small subset of patients receiving isotretinoin for acne are at risk for depression, which is very manageable provided there is adequate patient awareness of the possibility, maximum communication between the patient and physician, and cessation of therapy if clinically important depression occurs (after which the depression rapidly resolves in a week or less). Multiple controlled studies actually suggest a very favorable effect of isotretinoin on depression and anxiety common in the population requiring isotretinoin. With regard to inflammatory bowel disease, in just one study, only ulcerative colitis association with isotretinoin reached statistical significance. The actual incidence of this association is strikingly low. Finally, it is clear that even the most recent pregnancy prevention program (iPledge) is no more successful than prior programs; there will likely always be a small number of female patients becoming pregnant while receiving isotretinoin for acne vulgaris.

Immunosuppression and sebaceous tumors: a confirmed diagnosis of Muir-Torre syndrome unmasked by immunosuppressive therapy.

Muir-Torre syndrome (MTS) is an autosomal dominant genodermatosis caused by mutations in the DNA mismatch repair genes MLH1 and MSH2. This case describes a patient with an extensive family history of colon cancer who experienced the onset of multiple sebaceous adenomas and carcinomas after undergoing kidney transplantation and receiving immunosuppressive therapy. The finding of deficient MSH2 expression in the immunohistochemical analysis of a sebaceous carcinoma prompted genetic testing for a systemic mutation in the mismatch repair gene. A systemic mutation of the MSH2 gene was detected and, despite the absence of a visceral malignancy, the diagnosis of MTS was made. Immunosuppression has previously been thought to play a possible role in unmasking a latent MTS phenotype in transplant recipients, but systemic mutations have not previously been analyzed. The relationship between immunosuppression and sebaceous tumors with the possibility of unmasking a MTS phenotype in transplant recipients is discussed.

Recent trends in systemic psoriasis treatment costs.

OBJECTIVES: To analyze the current total cost of systemic therapy for psoriasis and to compare annual trends in the cost of both generic and brand-name therapies with trends in the Consumer Price Index-Urban since 2000. DESIGN: A cost model was developed that includes costs for prescription drugs, office visits, and suggested laboratory tests and monitoring procedures. Annual trends in psoriasis drug costs from 2000 through 2008 were analyzed by calculating the percentage change in the average wholesale price from the previous year; these values were compared with changes in the yearly Consumer Price Index-Urban values. SETTING: The United States. MAIN OUTCOME MEASURES: Total annual costs for systemic psoriasis therapies and trends in cost compared with the trends in Consumer Price Index-Urban values (equivalent to inflation). RESULTS: Current total annual costs for systemic psoriasis therapies ranged from $1197 (methotrexate) to $27,577 (alefacept, two 12-week courses). Trends in the average wholesale price of brand-name psoriasis therapies from 2000 through 2008 demonstrate an average increase of 66% (range, -24% to +316%); thus, costs of several brand-name psoriasis drugs greatly outpaced the rates of inflation for all items and all prescription drugs. CONCLUSIONS: Despite the higher monitoring costs associated with traditional systemic therapies, annual costs of biologics exceed those of other available therapies. Current trends demonstrate that systemic psoriasis therapy costs are increasing at a much higher rate compared with general inflation.

Moxifloxacin-induced drug hypersensitivity syndrome with features of toxic epidermal necrolysis.

Moxifloxacin was recently reported to induce combined features of drug hypersensitivity syndrome (DHS) and toxic epidermal necrolysis (TEN). A simultaneous presentation of these two potentially life-threatening cutaneous drug eruptions with systemic features in the same patient is considered rare since they are probably induced by two separate mechanisms. There is only one previously reported case in which moxifloxacin was implicated in the induction of these combined drug hypersensitivity processes. This article presents the case of a 44-year-old Asian male who developed features of both TEN and DHS approximately one week after initial ingestion of moxifloxacin.

Can short courses of systemic corticosteroids truly cause osteonecrosis?

One of the most feared complications of long-term corticosteroid therapy is osteonecrosis (avascular necrosis, aseptic necrosis). There is, no doubt, a causal role for systemic corticosteroids inducing osteonecrosis with such chronic therapy. The controversy involves whether short-term (<1 month) courses of systemic corticosteroids can truly induce osteonecrosis. This article presents both the biologic basis and statistical support for why such short-term courses of systemic corticosteroids rarely, if ever, truly induce osteonecrosis. Data from two very large populations (renal transplantation and systemic lupus erythematosus) with overall increased risk for osteonecrosis are carefully examined in view of the aforementioned controversy.

The efficacy and safety of rituximab in refractory pemphigus: a review of case reports.

Rituximab is a chimeric murine-human monoclonal antibody that targets the CD20 antigen found on B cells and results in rapid depletion of this cell population. It is indicated for patients with relapsed or refractory, low-grade or follicular, CD20-positive, B cell non-Hodgkin's lymphoma. In addition, rituximab has been used for many other diseases, including refractory pemphigus. In this study, 42 case reports of patients with refractory pemphigus vulgaris, pemphigus foliaceus, and paraneoplastic pemphigus treated with rituximab were reviewed for clinical efficacy and safety. Forty-one of the 42 patients had at least some improvement following the rituximab therapy, while 6 suffered infectious adverse events. Though rituximab appears to be effective in the treatment of refractory pemphigus diseases, further studies are warranted to clarify its overall safety, especially concerning the risk of infectious adverse events in this patient population.

Suggested guidelines for patient monitoring: hepatic and hematologic toxicity attributable to systemic dermatologic drugs.

Hepatic and hematologic toxicity are among the most fearful adverse effects that occasionally occur as a result of systemic drugs in the dermatologist's therapeutic armamentarium. Drugs of greatest interest concerning hepatic toxicity include methotrexate, azathioprine, dapsone, and acitretin. Somewhat overlapping are drugs that have important hematologic toxicities, including methotrexate, azathioprine, dapsone, sulfonamides, cyclophosphamide, and chlorambucil. Laboratory tests most commonly used include (1) hepatic monitoring: transaminases (AST/SGOT and ALT/SGPT) and the ultrasound-guided liver biopsy, and (2) hematologic monitoring: CBC with diff and platelets along with occasional use of the reticulocyte count. Important principles and specific guidelines for monitoring by drug group are highlighted.

Pemphigus foliaceus with epidermal detachment: adverse events from patch testing.

Uncommon adverse reactions to patch testing have been reported, but few cases have shown patch testing to be a potential contraindication. We report a patient with known pemphigus foliaceus who had significant epidermal detachment of normal skin during the removal of patch-testing tape.