Erin E. Boh

Cutaneous Reactions to Chemotherapeutic Agents

The task of evaluating a cutaneous eruption in the patient receiving chemotherapy can be quite formidable. Most of the time, these patients are receiving a multitude of agents and have profound immunosuppression. These factors may alter the more common manifestations of cutaneous eruptions. This article presents some of the more common cutaneous eruptions that may occur in an oncology patient receiving chemotherapy. It is hoped we may recognize clinical patterns seen with chemotherapeutic agents in the immunosuppressed population and, by recognizing these cutaneous eruptions, we may avoid the pitfalls of discontinuing medicines that may certainly be needed or altering the treatment course in a patient.

Role of Borrelia burgdorferi in the pathogenesis of morphea/scleroderma and lichen sclerosus et atrophicus: a PCR study of thirty‐five cases

Morphea (localized scleroderma), and lichen sclerosus et atrophicus (LSA) share common features with acrodermatitis chronica atrophicans (ACA), a known chronic form of borreliosis. These include similar histologic findings such as diffuse dermal fibrosis. These observations have led several investigators to consider the possibility of Borrelia burgdorferi (Bb) as a common etiologic factor among all of these diseases.

Propylthiouracil hypersensitivity: Report of two patients with vasculitis and review of the literature

Two patients with a hypersensitivity vasculitis in association with propylthiouracil (PTU) administration are described. Although both patients presented with a cutaneous eruption, our first patient suffered severe systemic manifestations and the second patients involvement was primarily limited to the skin. Patients with a vascular hypersensitivity reaction to PTU typically present with constitutional symptoms, acral purpuric skin lesions, and variable involvement of multiple organ systems. The reaction is treated by urgent withdrawal of PTU and implementation of supportive measures and immunosuppressive agents, as necessary. Prompt recognition of this condition and initiation of appropriate therapy lead to complete recovery in most cases.

Use of etanercept for psoriatic arthritis in the dermatology clinic: The Experience Diagnosing, Understanding Care, and Treatment with Etanercept (EDUCATE) study

Objective: To assess the efficacy and tolerability of etanercept to treat psoriatic arthritis. Materials and methods: A total of 1122 patients who had active psoriatic arthritis were enrolled in a Phase 4, non‐randomized, open‐label, single‐arm, 24‐week study. These patients had clinically stable, plaque psoriasis involving ⩾10% body surface area and joint disease (either ⩾two swollen and ⩾two tender/painful joints for ⩾3 months, or ⩾one joint with sacroiliitis or spondylitis). They received etanercept therapy 50 mg subcutaneously once weekly for 24 weeks. Results: After 24 weeks of treatment, 865 patients (77.1%; 95% CI: 74.64–79.55%) achieved a ‘mild or better’ score on the physician global assessment of psoriasis and were improved from baseline. Mean improvement in body surface area involvement was 16.9 percentage points (15.89–17.91). Patient global assessment of psoriasis, joint pain, and joint disease scores were improved by means of 2.2 (2.15–2.34), 2.7 (2.53–2.84), and 1.5 (1.39–1.55), respectively. Thirty‐five patients (3.1%) experienced at least one serious adverse event. No patient died during the study. Conclusions: These results support the effectiveness and tolerability of etanercept treatment in patients with psoriatic arthritis being treated at dermatology clinics.

Cutaneous manifestations of gastrointestinal diseases

Prompt recognition of cutaneous diseases or manifestations associated with the gastrointestinal tract may be lifesaving at times, and may lead to early preventive intervention to decrease risk of malignancy.

Epidermolysis bullosa acquisita preceding the development of systemic lupus erythematosus

In most cases of epidermolysis bullosa acquisita that occur in patients with systemic lupus erythematosus, the diagnosis of systemic lupus erythematosus is made before the development of blistering. We observed three patients with well-documented epidermolysis bullosa acquisita that developed several years before the onset of systemic lupus erythematosus. One patient was producing anti-U1RNP autoantibodies at the time epidermolysis bullosa acquisita was diagnosed, and all five produced this antibody during the systemic lupus erythematosus phase of their illness. In addition, in all five cases of epidermolysis bullosa acquisita with systemic lupus erythematosus antibodies to double-stranded DNA ultimately developed, and severe systemic lupus erythematosus and lupus nephritis developed in four patients. Sera from 15 other patients with epidermolysis bullosa acquisita without overt systemic lupus erythematosus were analyzed for systemic lupus erythematosus-related autoantibodies. Four patients were found to have at least one such autoantibody. These findings further document an association between epidermolysis bullosa acquisita and systemic lupus erythematosus and suggest that patients with systemic lupus erythematosus who present with epidermolysis bullosa acquisita may represent a subset of lupus erythematosus that puts the patient at increased risk for the development of more severe systemic illness. Patients presenting with epidermolysis bullosa acquisita, especially those who are black or Hispanic, should be monitored for the development of potentially life-threatening systemic lupus erythematosus.

From the Medical Board of the National Psoriasis Foundation: Treatment targets for plaque psoriasis

Background: An urgent need exists in the United States to establish treatment goals in psoriasis. Objective: We aim to establish defined treatment targets toward which clinicians and patients with psoriasis can strive to inform treatment decisions, reduce disease burden, and improve outcomes in practice. Methods: The National Psoriasis Foundation conducted a consensus‐building study among psoriasis experts using the Delphi method. The process consisted of: (1) literature review, (2) pre‐Delphi question selection and input from general dermatologists and patients, and (3) 4 Delphi rounds. Results: A total of 25 psoriasis experts participated in the Delphi process. The most preferred instrument was body surface area (BSA). The most preferred time for evaluating patient response after starting new therapies was at 3 months. The acceptable response at 3 months postinitiation was either BSA 3% or less or BSA improvement 75% or more from baseline. The target response at 3 months postinitiation was BSA 1% or less. During the maintenance period, evaluation every 6 months was most preferred. The target response at every 6 months maintenance evaluation is BSA 1% or less. Limitations: Although BSA is feasible in practice, it does not encompass health‐related quality of life, costs, and risks of side effects. Conclusion: With defined treatment targets, clinicians and patients can regularly evaluate treatment responses and perform benefit‐risk assessments of therapeutic options individualized to the patient.

Atypical cutaneous lymphocytic infiltrate and a role for quantitative immunohistochemistry and gene rearrangement studies.

Aim To help clarify the significance of the T‐cell receptor (TCR) gene rearrangement and its relationship to the immunophenotyping of histologically atypical cutaneous T‐cell lymphoid infiltrates (ACLIs).

Treatment of refractory necrobiotic xanthogranulomas with extracorporeal photopheresis and intravenous immunoglobulin

Necrobiotic xanthogranuloma (NXG) is a disease of fibrotic or telangiectatic granulomatous papules and nodules that can ultimately progress into ulcerated plaques. Although the exact cause of NXG is unknown, it most often occurs in patients with paraproteinemia secondary to a hematologic disease. Consequently, therapy for NXG is targeted at treating the underlying hematologic disease, and subsequent paraproteinemia, with alkylating agents, antimetabolites, radiation, and/or immunosuppressive agents. Cases refractory to these therapies often have poor outcomes. We report the successful treatment of two patients with refractory NXG with two different modalities: extracorporeal photopheresis (ECP) and intravenous immunoglobulin (IVIG). The first case shows a patient without paraproteinemia who had success with ECP and IVIG, and the second is a patient with paraproteinemia treated effectively with IVIG. The beneficial response of our patients to IVIG, as well as ECP, shows that they may be an effective treatment option for refractory NXG.

Etiologies and management of cutaneous flushing: Malignant causes

The second article in this 2-part continuing medical education series reviews the following malignant causes of flushing: mastocytosis, medullary thyroid carcinoma, pheochromocytoma, carcinoid tumors, gastroenteropancreatic neuroendocrine tumors, bronchogenic carcinoma, vasointestinal polypeptide secreting tumors, and renal cell carcinoma. The information provided will allow physicians to better distinguish patients who have worrisome presentations that require a more thorough investigation. Appropriate diagnostic workup and treatment options for these malignancies are reviewed.