Kenneth J. Tomecki

Wegener's granulomatosis: Cutaneous and oral mucosal disease

BACKGROUND Wegeners granulomatosis is a complex disease that can be difficult to diagnose, especially if the classic triad of necrotizing granulomas of the respiratory tract, generalized vasculitis, and glomerulitis are not present. A delay in diagnosis may result in irreversible kidney damage and ultimately death. OBJECTIVE This study was done to identify clinical and histologic findings of the skin and oral mucosa that may aid in the diagnosis of this disease. METHODS The records of 40 patients with Wegeners granulomatosis were reviewed. RESULTS Of these patients 12 had cutaneous lesions, 2 had oral mucosal lesions, and 2 had both cutaneous and oral mucosal lesions. Cutaneous and oral mucosal lesions were a presenting sign in 10% and 5% of patients, respectively. Overall, 25% demonstrated at least one of the specific cutaneous histopathologic findings described for Wegeners granulomatosis, that is, necrotizing vasculitis, granulomatous vasculitis, and palisading granuloma. One patient had gingival hyperplasia with petechiae, considered to be pathognomonic for Wegeners granulomatosis. CONCLUSION This study demonstrates that cutaneous or oral mucosal findings may serve as valuable diagnostic aids when evaluating a patient suspected of having Wegeners granulomatosis.

First 500,000 AIDS cases—United States, 1995

As of October 31, 1995, a total of 501,310 persons with acquired immunodeficiency syndrome (AIDS) have been reported to CDC by state and territorial health departments; 311,381 (62%) had been reported as having died. The AIDS surveillance case definition was substantially expanded in late 1987 and again in 1993 to reflect increased knowledge of the natural history of human immunodeficiency virus (HIV) and to remain consistent with the clinical management of HIV disease (1,2). This report presents rates of reported AIDS cases for 1994 and describes the temporal changes in the characteristics of persons reported with AIDS during three periods corresponding to changes in the AIDS case definition--1981-1987, 1988-1992, and 1993-October 1995--and how this information can be used to plan local, state, and national prevention programs.

Case report and review of resolved fusariosis

Erythematous macules, nonpalpable and palpable purpura, and flaccid pustules developed in a 59-year-old man with acute lymphocytic leukemia 8 days after reinduction chemotherapy with cytosine arabinoside and daunorubicin. Tissue and blood cultures grew Fusarium proliferatum, and a skin biopsy specimen revealed fungal vasculitis. Anemia and muscle weakness accompanied the disseminated infection, for which the patient received granulocyte transfusions and amphotericin B, ketoconazole, rifampin, and griseofulvin. Skin lesions and fungemia resolved with recovery of the bone marrow, and 51 days after the completion of his chemotherapy he returned home. If promptly recognized and aggressively treated, disseminated fusariosis is responsive to therapy. Infection with Fusarium species should be suspected in profoundly neutropenic patients in whom disseminated palpable purpura and myositis develop concomitantly.

Necrolytic migratory erythema : a report of three cases

Necrolytic migratory erythema (NME) is a cutaneous reaction pattern with specific histopathologic features that is typically associated with a functioning pancreatic islet cell neoplasm such as a glucagonoma. Three examples of NME, each associated with a different cause, are presented: glucagonoma, pancreatic insufficiency, and gluten-sensitive enteropathy. All three patients were successfully treated by surgical resection of the pancreatic tumor, total parenteral nutrition and pancreatic enzyme replacement, or a strict gluten-free diet, respectively. All remain free of skin disease more than 2 years later. Any patient with NME should be evaluated for glucagonoma and small bowel disease that may be associated with malabsorption and malnutrition.

Tick-borne infections

Ticks, obligate, blood-sucking members of the order Acarina and class Arachnida, are the most common agents of vector-borne diseases in the United States. Ticks play an important role in transmitting viruses, bacteria, spirochetes, parasites, and rickettsia. This article reviews the epidemiology, microbiology, diagnosis, and treatment of the major tick-borne diseases in the United States.

Dermatitis as a characteristic phenotype of a new autoinflammatory disease associated with NOD2 mutations

OBJECTIVES We sought to characterize a new category of autoinflammatory disease associated with nucleotide-binding oligomerization domain 2 (NOD2) gene mutations. METHODS A total of 22 patients were identified, inclusive of those reported previously. All had autoinflammatory phenotypes and NOD2 gene mutations that were prospectively studied between January 2009 and February 2012. RESULTS All 22 patients were non-Jewish whites (13 women and 9 men). The mean age at diagnosis was 40.1 years (range 17-72), with a mean disease duration of 4.7 years (range 1-13). Three female patients were siblings. Common clinical features were weight loss (13/22), episodic self-limiting fever (13/22), dermatitis (19/22), and inflammatory polyarthritis/polyarthralgia (20/22). Gastrointestinal symptoms occurred in 13 patients, sicca-like symptoms in 9, and recurrent chest pain in 5. All patients carried the NOD2 gene mutations, with the intervening sequence 8(+158) variant in 21 and the R702W variant in 8. LIMITATIONS The NOD2 allelic frequency may need to be examined in a larger population with systemic autoimmune diseases. CONCLUSIONS The characteristic clinical phenotype, notably dermatitis, coupled with certain NOD2 variants constitutes a new autoinflammatory disease entity, which we have named as NOD2-associated autoinflammatory disease.

Bullous Systemic Lupus Erythematosus

ABSTRACT: Two patients with bullous systemic lupus erythematosus are reported. In one, the disease appears to have been caused by hydralazine. The cutaneous lesions of bullous SLE have not been previously reported in drug‐induced lupus.

UV protection and sunscreens: what to tell patients.

Ultraviolet (UV) radiation is the major environmental risk factor for nonmelanoma skin cancer and is a suspected risk factor for melanoma. Avoiding overexposure to direct sunlight during the peak daylight hours, wearing protective clothing, and applying sunscreen are ways to protect the skin. To provide clinicians with the tools to advise patients and to answer their inquiries, including which sunscreen to use, we review UV radiation’s effect on the skin, how sunscreens block UV light, current recommendations on sunscreen use, and new sunscreen labeling requirements. Some measures are straightforward, but sunscreens have been a source of confusion in their labeling and SPF ratings.

CUTANEOUS (NON-HIV) INFECTIONS

Cutaneous infections continue to represent a large proportion of inpatient dermatology. Though most infectious skin diseases do not warrant hospitalization, some do and can rapidly become fatal if not treated promptly. A selected group of infections are reviewed--primary cutaneous infections, exotoxin-mediated syndromes, and systemic infections--that warrant hospitalization. Dermatologists play a critical role in the synthesis of patient history and appreciation of morphologic skin disease, which, when coupled with appropriate lab tests, may help to establish a diagnosis allowing for the timely implementation of effective and targeted therapy.

Tinea Capitis in Three Adults

Abstract: Three cases of tinea capitis in healthy adults are presented. In light of the very low incidence (<1%) of tinea capitis in nonimmunocompromised adults and the possibility of contagion from asymptomatic dermatophyte carriers in the pediatric and adult populations, the necessity for heightened clinical suspicion and diagnostic tenacity in the evaluation of adults with scalp dermatitis and/or alopecia for possible tinea capitis is underscored.