Bonnie T. Mackool

A 10-year experience with toxic epidermal necrolysis

Toxic epidermal necrolysis is a devastating medication-induced desquamation disorder with a reported mortality rate of 30% to 60% in adults. Data from previously reported series suggest that age, delay in referral to a burn center, total body surface area (TBSA) involvement, and systemic steroid treatment are poor prognostic indicators. We reviewed the records of 39 patients treated in our burn center over the past 10 years and found that the mortality rate was significantly correlated with age, thrombocytopenia, and delay in presentation. Steroid treatment and TBSA involvement were not significantly related to the mortality rate. Thirty-nine adult patients with greater than 20% TBSA epithelial necrosis were cared for in our center from January 1987 to March 1998. Wounds were treated with topical antimicrobial medications and porcine xenografts in a bacteria-controlled nursing unit. We reviewed the records of these patients for 28 clinical characteristics and looked for clinical correlates of mortality by single analysis of variance. The mortality rate was 44% (17 of 39 patients); the cause of death was most commonly multiple-organ dysfunction syndrome, for which a microbial etiologic agent was not always identified. Autopsies were performed on 11 of the 17 patients who died; there was evidence of multiple-organ damage. The patients who survived and the patients who died did not differ significantly in TBSA epithelial necrosis (66%+/-6% vs 72%+/-5%, respectively), admission platelets, number of nosocomial infections, number of complications, preadmission exposure to steroids, or extent of mucosal involvement. When compared with the patients who died, the patients who survived were (1) 20 years younger (47.5+/-4.2 years vs 64.5+/-5.3 years), (2) admitted to the hospital sooner after the onset of their rash (3.5+/-0.4 days vs 5.9+/-1.0 days), (3) much less likely to experience early thrombocytopenia (platelet nadir, 154+/-24 vs 70+/-18), (4) more likely to be febrile on presentation, and (5) less likely to have been treated with antibiotics before referral to our unit. These differences were statistically significant. The most common etiologic agents were antibiotics, anticonvulsants, and nonsteroidal anti-inflammatory drugs. Our results for a group of older patients with toxic epidermal necrolysis with extensive skin involvement suggest that age, delay in hospitalization, thrombocytopenia, and early empiric antibiotic treatment are associated with a poor prognosis.

Pityriasis rubra pilaris

Pityriasis rubra pilaris (PRP) is an idiopathic, papulosquamous disease which has remained enigmatic for more than one and a half centuries after its first description. A review of the clinical features, histopathologic findings, classification, and treatment is presented. Subjects of recent focus in the literature are discussed, including the occurrence of PRP in patients with human immunodeficiency virus (HIV), speculation that PRP may result from an abnormal immune response to antigenic stimulation, and additions to the histopathologic descriptions of the disease.

Mycophenolate in dermatology

Originally used to treat psoriasis nearly three decades ago, mycophenolic acid, reformulated as mycophenolate mofetil (MMF), has been rediscovered by the world of dermatology. As a relatively well‐tolerated immunosuppressive used in organ transplant recipients, MMF has recently been reported to show promise for several dermatologic conditions, including psoriasis, pemphigus vulgaris, pyoderma gangrenosum, bullous lichen planus, and even connective tissue diseases such as lupus erythematosus and dermatomyositis. Although not intended to be exhaustive, this review discusses MMF with regard to its basic pharmacology, its side effects, and its reported efficacy in a variety of dermatologic indications. Relevant literature was retrieved by a Medline search combining the terms ‘mycophenolate’ or ‘mycophenolic acid’ and ‘skin’ or ‘skin disease’ or a number of specific conditions (‘psoriasis’, ‘dermatitis’, ‘eczema’, ‘pemphigoid’, ‘pemphigus’, ‘vasculitis’, ‘pyoderma gangrenosum’, ‘Crohns disease’, ‘graft‐versus‐host disease’, ‘lichen planus’). As MMF has only been recently re‐introduced for dermatologic application, the nature of much of the literature is admittedly that of case reports or case series. Nevertheless, the results are sufficiently promising to warrant further larger, control studies.

Case records of the Massachusetts General Hospital. Weekly clinicopathological exercises. Case 35-2004. A 68-year-old man with end-stage renal disease and thickening of the skin.

From the Department of Dermatology, Lahey Clinic, Burlington, Mass. (S.L.M.); the Division of Rheumatology, Department of Medicine (J.K.), the Department of Dermatology (B.T.M.), and the Division of Dermatopathology, Department of Pathology (V.L.), Massachusetts General Hospital; and the Departments of Dermatology (S.L.M., B.T.M.), Medicine (J.K.), and Pathology (V.L.), Harvard Medical School.

Vancomycin-induced linear IgA disease manifesting as bullous erythema multiforme.

Background:  Vancomycin‐induced linear immunoglobulin A (IgA) disease, an autoimmune, blistering disease in response to vancomycin administration, is characterized by a subepidermal, vesiculobullous eruption and linear IgA deposition along the basement membrane zone on direct immunofluorescence.

Case 35-2004: A 68-Year-Old Man with End-Stage Renal Disease and Thickening of the Skin

From the Department of Dermatology, Lahey Clinic, Burlington, Mass. (S.L.M.); the Division of Rheumatology, Department of Medicine (J.K.), the Department of Dermatology (B.T.M.), and the Division of Dermatopathology, Department of Pathology (V.L.), Massachusetts General Hospital; and the Departments of Dermatology (S.L.M., B.T.M.), Medicine (J.K.), and Pathology (V.L.), Harvard Medical School.

Management of airway obstruction in cicatricial pemphigoid

Cicatricial pemphigoid is a chronic vesiculobullous disease of the mucosal epithelium that primarily involves the oral cavity and the eyes. The clinical and histologic features are identical to those of bullous pemphigoid, and these features often can be nonspecific for other disease processes. It is not unusual for a period of 1 year or more to elapse before a diagnosis is made. The diagnosis of cicatricial pemphigoid requires characteristic lesions and histopathologic evidence of immunoglobulin deposition along the basement membrane, as well as a high index of suspicion. The authors detail a case of cicatricial pemphigoid resulting in airway obstruction and present the treatment required for both stabilization of the airway and resolution of the disease process.

Case 35-2004

From the Department of Dermatology, Lahey Clinic, Burlington, Mass. (S.L.M.); the Division of Rheumatology, Department of Medicine (J.K.), the Department of Dermatology (B.T.M.), and the Division of Dermatopathology, Department of Pathology (V.L.), Massachusetts General Hospital; and the Departments of Dermatology (S.L.M., B.T.M.), Medicine (J.K.), and Pathology (V.L.), Harvard Medical School.

Case records of the Massachusetts General Hospital: Case 7-2011: a 52-year-old man with upper respiratory symptoms and low oxygen saturation levels.

Dr. Roby P. Bhattacharyya (Medicine): A 52-year-old man was seen in the urgent care outpatient medical clinic at this hospital because of upper respiratory symptoms. The patient had been in his usual state of health until 3 days earlier, when subjective fever, fatigue, headache, nasal and sinus congestion, sore throat, and a nonproductive cough developed. Three days later, he came to the outpatient clinic. He did not have chills, gastrointestinal symptoms, shortness of breath, wheezing, night sweats, or chest discomfort, and he reported that his respiratory symptoms were not as severe as those he had had during a previous episode of pneumonia. The patient had had type 1 diabetes mellitus for 17 years, for which he was followed at another hospital. Control of blood glucose levels had been poor, despite low hemoglobin A1c measurements; glycemic control was followed by measuring the levels of plasma fructosamine. Eight years before this evaluation, he had an episode of prolonged altered consciousness and somnolence, associated with a fasting blood sugar level of 49 mg per deciliter (2.7 mmol per liter); his condition improved slowly after the administration of glucagon and intravenous glucose. Eighteen months before this evaluation, he had an episode of transient expressive aphasia and was treated briefly with valproic acid. He also had hypertension, exercise-induced angina, hyperlipidemia, hyperbilirubinemia of more than 8 years’ duration, glaucoma, and dermatitis herpetiformis, for which he had taken dapsone for more than 8 years. Testing for serum endomysial antibodies had been positive, and he had briefly tried a gluten-free diet but had not adhered to it. He had migraine headaches, atypical chest pains, and peripheral neuropathy with mildly diminished vibratory sensation in his feet. Episodes of elevated aminotransferase levels had occurred 18 months, 8 months, and 2 months before this evaluation, after the initiation of valproic acid, simvastatin, and atorvastatin, respectively. A screening colonoscopy had revealed diverticulosis and a tubular adenoma (4 mm in diameter), which had been excised. Medications included the human insulin analogue lispro, insulin glargine, dapsone (200 mg daily), lisinopril, metoprolol succinate, and nitroglycerin as needed for chest pain. The patient reported omitting his antihypertensive medications for the 3 days preceding the current evaluation, and he had not taken Case 7-2011: A 52-Year-Old Man with Upper Respiratory Symptoms and Low Oxygen Saturation Levels

Case 14-2012 : A 43-Year-Old Woman with Fever and a Generalized Rash

Dr. Sarah Gee (Dermatology): A 43-year-old woman was admitted to the hospital because of fever and a generalized rash. The patient had a history of atopic dermatitis and had been in her usual health until approximately 1 week before admission, when a painful, pruritic rash developed on her chin, neck, and chest; it gradually spread to involve her entire body, sparing the palms, soles, and genitalia. Four days before admission, she went to the emergency department at another hospital. She reported that the rash was similar to but more severe than her usual atopic dermatitis, which was not painful. Prednisone and hydroxyzine were administered, and she was discharged home. The next day, the rash persisted, chills developed, and the temperature reportedly rose to 39.4°C. The patient returned to the other hospital. On examination, the temperature was 38.6°C, and the blood pressure, pulse, and oxygen saturation were normal. A diffuse, erythematous rash with vesicles, pustules, and excoriations involved the forehead, nose, lips, chin, periorbital areas, chest, back, arms, and legs and spared the palms, soles, and part of the cheeks. The complete blood count was reportedly normal. The patient was admitted to the other hospital, and clindamycin, hydromorphone, hydroxyzine, acetaminophen, and intravenous fluids were administered; white petrolatum ointment was applied to dry and cracking areas. The next day, a dermatologic consultation was obtained, and a skin biopsy and culture were performed. A chest radiograph was normal. During the second night, the temperature rose to 39.6°C, and sloughing of the skin occurred with pressure. Gram’s staining of the skin-biopsy specimen reportedly showed no neutrophils or organisms; culture of the skin revealed a heavy growth of Staphylococcus aureus. Infectious disease consultation was obtained; the administration of clindamycin was stopped, and vancomycin and acyclovir were begun. The blood pressure ranged from 110 to 130 mm Hg systolic and from 50 to 69 mm Hg diastolic, and the oxygen saturation was 95 to 100% while the patient was breathing ambient air. Blood cultures were sterile. Other laboratory-test and culture results were pending. The patient was transferred to this hospital. Medications on transfer included vancomycin, acyclovir, hydromorphone, hydroxyzine, acetaminophen, and fluoxetine, with albuterol as needed. Case 14-2012: A 43-Year-Old Woman with Fever and a Generalized Rash