Alison J. Bruce

Urban legends: recurrent aphthous stomatitis.

Recurrent aphthous stomatitis (RAS) is the most common idiopathic intraoral ulcerative disease in the USA. Aphthae typically occur in apparently healthy individuals, although an association with certain systemic diseases has been reported. Despite the unclear etiopathogenesis, new drug trials are continuously conducted in an attempt to reduce pain and dysfunction. We investigated four controversial topics: (1) Is complex aphthosis a mild form of Behçets disease (BD)? (2) Is periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome a distinct medical entity? (3) Is RAS associated with other systemic diseases [e.g., celiac disease (CD) and B12 deficiency]? (4) Are there any new RAS treatments? Results from extensive literature searches, including a systematic review of RAS trials, suggested the following: (1) Complex aphthosis is not a mild form of BD in North America or Western Europe; (2) Diagnostic criteria for PFAPA have low specificity and the characteristics of the oral ulcers warrant further studies; (3) Oral ulcers may be associated with CD; however, these ulcers may not be RAS; RAS is rarely associated with B12 deficiency; nevertheless, B12 treatment may be beneficial, via mechanisms that warrant further study; (4) Thirty-three controlled trials published in the past 6 years reported some effectiveness, although potential for bias was high.

Neutrophilic dermatosis of the hands: four new cases and review of the literature

Background  Isolated or predominantly hand involvement in Sweets syndrome, pyoderma gangrenosum, or pustular vasculitis is a rare presentation in the spectrum of neutrophilic dermatoses and is often associated with an occult malignancy or other systemic inflammatory disorder. When these disorders occur on the hands, they are often clinically indistinguishable, but they can sometimes be separated histologically by the presence of papillary dermal edema (Sweets syndrome), ulceration and necrosis (pyoderma gangrenosum), or vasculitis (pustular vasculitis). These distinctions may be arbitrary, however, and reflect differences in the temporal course of the disease and in the degree of inflammation at the time of biopsy.

Acute oral ulcers

In general, a detailed history and examination of the patient provides sufficient information for diagnosis. The pattern, frequency, and natural history of ulcer episodes are helpful. The presence or absence of associated features and the site of oral involvement guides most physicians accurately in the diagnosis. Additional investigations, including blood tests, and occasionally the use of oral cultures or biopsy, are needed to make a definitive diagnosis. A multispecialty approach is often necessary to evaluate patients with other systemic features. Most acute oral ulcers heal spontaneously without specific therapy being necessary, but an understanding of the cause of the ulcer is reassuring to the patient and guides the clinician in management to prevent recurrent episodes of oral ulceration, or chronicity of ulcers.

Effectiveness of Intravenous Immunoglobulin Therapy for Skin Disease Other Than Toxic Epidermal Necrolysis: A Retrospective Review of Mayo Clinic Experience

OBJECTIVE To examine retrospectively the use and effectiveness of intravenous immunoglobulin (IVIg) treatment of various skin diseases, primarily immunobullous disease. PATIENTS AND METHODS We identified patients who had received IVIg therapy for skin disease between 1996 and 2003 at the Mayo Clinic in Rochester, Minn, Scottsdale, Ariz, and Jacksonville, Fla, and retrospectively reviewed their medical records. RESULTS Eighteen patients were treated with IVIg for various skin diseases: immunobullous disease in 11 adults (pemphigus vulgaris [7 patients], bullous pemphigold [3], and cicatricial pemphigoid [1]); dermatomyositis (2); mixed connective tissue disease (1); chronic urticaria (1); scleromyxedema (1); leukocytoclastic vasculitis (1); and linear IgA bullous disease (1). Responses of patients by type of disease were as follows: pemphigus vulgaris, 1 partial response (PR) and 6 no response (NR); bullous pemphigoid, 1 complete response (CR) and 2 NR; cicatricial pemphigoid, 1 NR; dermatomyositis, 1 CR and 1 PR; mixed connective tissue disease, 1 CR; chronic urticaria, 1 CR; scleromyxedema, 1 CR; leukocytoclastic vasculitis, 1 PR; and linear IgA bullous disease, 1 CR. Six patients (33%) experienced CR, 3 (17%) had PR, and 9 (50%) had NR to IVIg therapy. All 9 nonresponders were adult patients with immunobullous disease. CONCLUSION Although this was a retrospective study of a small cohort of a mixture of patients, the findings emphasize that our experience with IVIg treatment for skin disease, particularly immunobullous disease, is less favorable than that reported previously. Further studies are needed to verify the efficacy of IVIg for skin disease.

Variations in Presentations of Esophageal Involvement in Lichen Planus

BACKGROUND & AIMS Lichen planus is a relatively uncommon, presumed autoimmune disease that affects middle-aged patients and is manifested in the skin, nails, and mucous membranes. Reports of esophageal involvement have been rare, despite the classification of the esophagus as a mucous membrane. METHODS Assuming esophageal involvement might not be as rare as expected, we reviewed the Mayo Clinic Pathology Database for all cases from 2000 to the present. RESULTS Twenty-seven cases were identified, with a mean age of 64 years; 25 were women. Patients presenting with esophageal lichen planus as the initial manifestation and those with a diagnosis of lichen planus involving other sites were equal in number. Many patients had received multiple dilations and reflux treatments before diagnosis. All patients presented with dysphagia. Endoscopy and radiology studies demonstrated a wide range of abnormalities, including strictures of varying length and location, small-caliber esophagus, and a mucosal appearance of sloughing, white discoloration, erythema, thickening, and superficial ulceration. Treatment regimens varied markedly, with some patients responding to topical or systemic corticosteroids. CONCLUSIONS Esophageal lichen planus is rare but probably more common than previously suspected. It presents with a wide range of endoscopic signs and is commonly the presenting sign of lichen planus. In evaluating middle-aged patients with strictures, particularly proximal esophageal strictures in women, physicians should consider a diagnosis of lichen planus even in the absence of extraesophageal manifestations.

Successful Treatment of Complex Aphthosis With Colchicine and Dapsone

OBJECTIVE To investigate the effectiveness of colchicine and dapsone, 2 corticosteroid-sparing anti-inflammatory agents, in the treatment of patients with complex aphthosis (recurrent oral and genital aphthous ulcers or severe, almost constant, multiple oral aphthae in the absence of Behçet syndrome). DESIGN Retrospective review of medical records. SETTING Tertiary care medical clinic. PATIENTS Fifty-five patients with complex aphthosis evaluated and treated at Mayo Clinic between January 1, 1998, and July 31, 2007. All the patients were treated according to a therapeutic ladder, starting with colchicine and adding dapsone to treatment of patients who did not have a substantial response (>75% improvement) to colchicine or who discontinued colchicine use because of adverse effects. MAIN OUTCOME MEASURES A substantial response to therapy with colchicine alone, dapsone alone, or colchicine and dapsone combined. RESULTS Most patients (44 [80%]) had a substantial response to therapy and had no serious adverse effects. CONCLUSIONS Colchicine and dapsone are effective, safe therapies for the treatment of complex aphthosis. Colchicine and dapsone, 2 established drugs also used for gout and leprosy, respectively, and for other dermatologic disorders, should be considered efficacious in the treatment of complex aphthosis.

The management of mucous membrane pemphigoid and pemphigus

Mucous membrane pemphigoid and pemphigus vulgaris are autoimmune blistering disorders in which many similar drugs and therapeutic strategies are utilized. In general, localized disease can be treated with topical agents. In contrast, patients with more severe and progressive disease usually require a combination of systemic corticosteroids and immunosuppressive medications. Oral corticosteroids, adjuvant immunosuppressive agents, antibiotics such as dapsone and immunomodulatory procedures like intravenous immunoglobulin are the main therapeutic agents used in treating these two disorders. Much of the morbidity and mortality associated with these disorders are related to the sites involved and to the drugs used for therapy. Treatment should be individualized based on severity, extent, and rate of progression of disease, comorbidities, and age of the patient. Serum levels of specific autoantibodies and indirect immunofluorescence titers, in certain cases, can be used to monitor response to therapy.

The tongue in clinical diagnosis

The case report of Yucel et al . illustrates the importance of the tongue in clinical diagnosis. 1 The 53-year-old man suffered from macroglossia and purpura of the tongue and lips as a sign of myeloma-associated systemic amyloidosis. The woody, hard, enlarged tongue with purpura (fig. 1) is a classical sign of amyloidosis and a stimulus to seeking the cause for the amyloid deposition. Examination of the tongue can provide clinical clues to several conditions with systemic import as well as local abnormalities that may be recognized by the patient in self-examination. Recognition of tongue abnormalities as benign or as a sign of systemic disease is a valuable clinical skill. It is important for the clinician to know and recognize the spectrum of disorders affecting the tongue. A thorough examination of the tongue is an integral part of the physical examination and may provide clues to systemic disorders. A systematic approach to the examination, beginning with inspection of all aspects of the tongue (dorsum, lateral margins and ventral surface) and concluding with palpation of the entire surface from the tip proximally, provides a thorough and complete examination. Note the shape and colour of the tongue as it rests in the mouth. The presence of notching on the lateral margins of the tongue is indicative of macroglossia or a tongue-thrusting habit as the pressure from the teeth on the tongue creates the notches. The normal tongue is pink in colour, and the papillae easily visualized. Many conditions present with alterations in colour or texture of the tongue, and clinicians should be familiar with the normal appearance to best appreciate changes to normal architecture. 2,3

Glossitis and other tongue disorders

Patients frequently present complaining of tongue abnormalities. Knowledge of normal tongue anaomy an d architecture enable the clinician t odifferentiate variations of normal from abnormal conditions. Many tongue conditions are benign and. require reassurance and explanation, with little to no treatment. Others can signify systemic disorders. Examination of the tongue is an integral part of a complete physical examination. Recognizing the disorders of the tongue that are benign and do not require treatment or further evaluation prevents unnecessary testing for the patient. Careful evaluation of the tongue may provide valuable clues to a systemic disorder.

Overview of Skin Cancer Detection and Prevention for the Primary Care Physician

The incidence of both melanoma and nonmelanoma skin cancer is increasing, and an accurate and timely diagnosis is important to reduce the morbidity and mortality associated with these malignancies. Clinical features and examination techniques are discussed, and the need to recognize important secondary tumor characteristics, which supplement the primary diagnosis, is reviewed. Recent evidence on etiologic factors and their importance are addressed to highlight the role of primary prevention. Pediatricians, family physicians, and all primary care physicians are encouraged to promote skin cancer awareness among children and their parents.