Coexistence of oral lichen planus and vulvar lichen sclerosus.

OBJECTIVE: Oral lichen planus (OLP) is a mucosal variant of lichen planus. Lichen sclerosus (LS) is an inflammatory disorder with a predilection for genital skin. We aimed to identify the characteristics of patients with both mucosal diagnoses. STUDY DESIGN: This retrospective study included 86 women with both OLP and vulvar LS diagnosed from June 1, 1991 through November 30, 2020 at a Mayo Clinic campus in Rochester, Minnesota; Scottsdale, Arizona; or Jacksonville, Florida. Data included treatments, other cutaneous diagnoses, comorbidities, and information on patch testing and malignant transformation. RESULTS: The median patient age at diagnosis was 64.5 years for OLP and 65.6 years for vulvar LS. A diagnosis of OLP before vulvar LS was most common (50.0%). The most frequently used treatment for both conditions was topical corticosteroids. Oral squamous cell carcinoma (SCC) did not develop in any patient, but vulvar SCC developed in 2 (2.3%). CONCLUSIONS: OLP and vulvar LS may coexist, commonly beginning in the patient's seventh decade. Topical corticosteroids are often used to manage both conditions. The coexistence of both diseases did not seem to portend a greater malignancy risk.

Contact stomatitis.

Contact stomatitis occurs in up to 10% of the population. Mechanical or chemical irritation, ill-fitting dentures, and dental fillings can induce irritant contact stomatitis. Type I hypersensitivity and type IV hypersensitivity to dental products and foods are frequently responsible for the allergic types of contact stomatitis. We review the causal agents of contact stomatitis, the differential diagnoses, diagnostic testing, and potential treatment.

A Diagnostic Approach to Recurrent Orofacial Swelling: A Retrospective Study of 104 Patients.

OBJECTIVE: To identify patients evaluated in an outpatient setting at our institution with a presentation of recurrent orofacial swelling and to review the spectrum of causes to outline a diagnostic approach. PATIENTS AND METHODS: A retrospective study of 104 patients with more than 1 episode of orofacial swelling lasting for more than 5 days identified through a keyword search of the electronic health record from January 2, 2000, through July 5, 2011. RESULTS: Patients were categorized according to final cause of orofacial swelling: idiopathic orofacial granulomatosis, solid facial edema due to rosacea and acne vulgaris, Crohn disease, contact dermatitis, sarcoidosis, exfoliative cheilitis, lichen planus, actinic cheilitis, cheilitis glandularis, lymphedema, miscellaneous, and multifactorial. Granulomatous inflammation was noted on biopsy in 40 of 85 patients (47%). Oral involvement was associated with Crohn disease (P<.001), and facial and periorbital swelling was associated with solid facial edema in the setting of rosacea and acne vulgaris (P<.001). CONCLUSION: The broad range of diagnoses responsible for recurrent orofacial swelling underscores the diagnostic challenge and importance of a thorough multidisciplinary evaluation to identify underlying causes.

Acute oral ulcers.

Accurate diagnosis of acute oral ulcers can be challenging. Important historic details include the pattern of recurrence, anatomic areas of involvement within the mouth and elsewhere on the mucocutaneous surface, associated medical symptoms or comorbidities, and symptomology. Careful mucocutaneous examination is essential. When necessary, biopsy at an active site without ulceration is generally optimal. Depending on the clinical scenario, supplemental studies that may be useful include cultures; perilesional biopsy for direct immunofluorescence testing; and evaluation for infectious diseases, gluten sensitivity, inflammatory bowel disease, human immunodeficiency virus infection, connective tissue diseases, or hematinic deficiencies. Clinicians should maintain a broad differential diagnosis when evaluating patients with acute oral ulcers.

Recurrent aphthous stomatitis.

Recurrent aphthous stomatitis (RAS) is the most common acute oral ulcerative condition in North America. RAS is divided into a mild, common form, simple aphthosis, and a severe, less common form, complex aphthosis. Aphthosis is a reactive condition. The lesions of RAS can represent the mucosal manifestation of a variety of conditions. These include conditions with oral and genital aphthae such as ulcus vulvae acutum, reactive nonsexually related acute genital ulcers, and Behçet disease. The mouth is the beginning of the gastrointestinal (GI) tract, and the lesions of RAS can be a manifestation of GI diseases such as gluten-sensitive enteropathy, ulcerative colitis, and Crohn disease. Complex aphthosis may also have correctable causes. The clinician should seek these in a careful evaluation. Successful management of both simple and complex aphthosis depends on accurate diagnosis, proper classification, recognition of provocative factors, and the identification of associated diseases. The outlook for patients with both simple and complex aphthosis is positive.

Oral lichen planus.

Lichen planus is an inflammatory mucocutaneous disease that can affect the skin, hair, nails, and mucosal surfaces. Mucosal sites of involvement include oral, genital, ocular, otic, esophageal, and, less commonly, bladder, nasal, laryngeal, and anal surfaces. Oral lichen planus is a mucosal variant of lichen planus, which tends to affect women more often than men, with a typically more chronic course and potential for significant morbidity. Treatment can be challenging, and there is potentially a low risk of malignant transformation; however, therapeutic benefits can be obtained with various topical and systemic medications. Clinical monitoring is recommended to ensure symptomatic control. Increasing awareness and recognition of this entity have continued to fuel advances in therapy and in our understanding of the disease.

Diseases of the tongue.

The tongue is a complex organ involved in speech and expression as well as in gustation, mastication, and deglutition. The oral cavity, along with the tongue, are sites of neoplasms, reactive processes, and infections, and may be a harbinger of systemic diseases. This review includes both common and rare diseases that occur on the tongue, including: vascular and lymphatic lesions (infantile hemangiomas and oral varices), reactive and inflammatory processes (hairy tongue, pigmented fungiform papillae of the tongue, benign migratory glossitis, and fissured tongue), infections (oral hairy leukoplakia, herpes simplex and varicella-zoster virus infections, human papillomavirus, and candidiasis), premalignant lesions (leukoplakia and erythroplakia), malignant lesions (squamous cell carcinoma, Kaposi sarcoma, and lymphoproliferative diseases), and signs of systemic disease (nutritional deficiency and systemic amyloidosis).

Orofacial granulomatosis.

Orofacial granulomatosis (OFG) is an uncommon chronic granulomatous condition with a multifactorial etiology and pathogenesis. Genetic, immunologic, allergic, and infectious mechanisms have been implicated. OFG is often used as a descriptor to encompass all entities with orofacial swelling and histologic evidence of noncaseating granulomas. The diagnosis of OFG should prompt evaluation for provocative factors. The cause of most cases of OFG remains obscure. The clinician must consider mycobacterial infections, deep fungal infections, sarcoidosis, rosacea, and Crohn disease (CD). In addition, OFG should be considered synonymous with previously described conditions, including granulomatous cheilitis and Miescher cheilitis, as well as Melkersson-Rosenthal syndrome and its monosymtomatic or oligosymptomatic variants. The relationship of OFG to CD is currently debated, with recent evidence suggesting the possibility of three entities: classic oral CD (established gastrointestinal CD with oral involvement), OFG with gastrointestinal involvement (OFG with subclinical or asymptomatic gastrointestinal endoscopy changes), and OFG without bowel involvement. Childhood onset, presence of certain phenotypic features, laboratory abnormalities, or gastrointestinal symptoms may indicate the need for further evaluation to assess for concurrent or future CD. Although multiple therapies have been found to be effective in small numbers of patients, treatment should be tailored to each patient.

Clinical features of Behçet's disease: A retrospective chart review of 26 patients.

BACKGROUND: Behçet's disease (BD) is a multi-organ inflammatory disorder with mucocutaneous, ocular, neurological, musculoskeletal, gastrointestinal, and pulmonary manifestations. The aim of this study was to determine the clinical features of BD in a cohort of patients diagnosed at the University of California, Davis, United States. METHODS: The medical records of 59 patients with an ICD9-code of BD were retrospectively reviewed. The International Criteria for BD was used to identify patients with a point score ≥5. The clinical manifestations of BD were investigated over a 10-year period in this cohort. RESULTS: Twenty-six patients (20 women, six men; female:male ratio = 3.3:1.0) had a point score ≥5 based on the International Criteria. The average age of onset of BD was 29.25 ± 14.25 years. Oral ulcers (100%), genital ulcers (84.6%), articular involvement (69.2%), ocular involvement (65.3%), and skin lesions (50.0%) were the most prevalent manifestations of BD in this cohort. CONCLUSION: Oral ulcers were the most common presenting and lifetime sign of BD. In our cohort, oral and genital ulcers were hallmarks of BD, independent of patient demographics. Thus, patients with these signs require close surveillance for other manifestations of the disease.

Oral manifestations of connective tissue disease and novel therapeutic approaches.

Connective tissue diseases such as systemic lupus erythematosus (SLE), systemic sclerosis (SSc), and Sjögren syndrome (SS) have presented many difficulties both in their diagnosis and treatment. Known causes for this difficulty include uncertainty of disease etiology, the multitude of clinical presentations, the unpredictable disease course, and the variable cell types, soluble mediators, and tissue factors that are believed to play a role in the pathogenesis of connective tissue diseases. The characteristic oral findings seen with these specific connective tissue diseases may assist with more swift diagnostic capability. Additionally, the recent use of biologics may redefine the success rate in the treatment and management of the disease. In this review we describe the oral manifestations associated with SLE, SSc, and SS and review the novel biologic drugs used to treat these conditions.

Clinically relevant patch test results in patients with burning mouth syndrome.

BACKGROUND: Patients with a sore or burning mouth associated with clinically normal oral mucosa present a difficult diagnostic challenge. OBJECTIVE: The objective of this study was to assess the value of patch testing in patients with burning mouth syndrome. METHODS: We retrospectively reviewed the results of patch testing to an oral series in patients with burning mouth syndrome seen at Mayo Clinic, Rochester, Minnesota, between January 2000 and April 2006. RESULTS: Of 195 consecutive patients with a burning or sore mouth, 75 had patch testing to an oral series, and 28 of these patients (37.3%) had allergic patch test reactions. The most common allergens were nickel sulfate hexahydrate 2.5%, balsam of Peru, and gold sodium thiosulfate 0.5%. On follow-up, 15 patients reported improvement, 4 removed or avoided the offending dental metal, and 6 avoided the dietary allergen. Thirteen patients did not improve; 6 avoided identified allergens, but without improvement; 1 removed dental metals without symptom change; and 5 avoided test-positive dietary allergens but without improvement. The remaining 7 nonresponders had nonrelevant patch test results or did not avoid allergens. CONCLUSIONS: Patch testing can identify patients who may be allergic to dental metals or dietary additives and who may benefit from removal or avoidance of these.

Pemphigoid nodularis: a case report.

Several variants of bullous pemphigoid have been reported including pemphigoid nodularis. Patients with pemphigoid nodularis have clinical features of prurigo nodularis in combination with clinical or immunologic characteristics of bullous pemphigoid. We report the case of a 71-year-old woman with pemphigoid nodularis. The diagnosis was suspected clinically and established by positive indirect immunofluorescence (IIF) findings characteristic of pemphigoid. Results of direct immunofluorescence (DIF) testing were negative, which emphasizes the importance of conducting both DIF and IIF when pemphigoid nodularis is suspected.

Reactive nonsexually related acute genital ulcers: review of cases evaluated at Mayo Clinic.

BACKGROUND: Reactive nonsexually related acute genital ulcers (RNSRAGU) occur in pubertal girls after an acute systemic infection. OBJECTIVE: We sought to characterize RNSRAGU by reviewing the medical records of patients with this disorder. METHODS: We searched our medical index database from 1997 to 2007 for RNSRAGU cases. Questionnaires were mailed to identified patients. RESULTS: The study included 10 patients; 5 responded to the questionnaire. The mean age at onset was 11.5 years. Vulvar ulcers were preceded by viral gastroenteritis (n = 3), viral upper respiratory tract infection (n = 3), streptococcal pharyngitis (n = 1), influenza (n = 1), and other nonspecific febrile illnesses (n = 2). Seven patients had oral involvement also; 6 had at least one recurrence; and 3 were hospitalized for pain control. Analgesics and topical corticosteroids were the most common treatments. Ulcerations resolved within several weeks in all patients. LIMITATIONS: Retrospective study design, small study size, and 50% questionnaire response rate are limitations. CONCLUSIONS: Although rare, RNSRAGU should be considered when genital ulceration follows an acute systemic illness.

Thioguanine nucleotides and thiopurine methyltransferase in immunobullous diseases: optimal levels as adjunctive tools for azathioprine monitoring.

OBJECTIVE: To prospectively determine optimal levels of 6-thioguanine nucleotide for disease remission in patients with immunobullous disease treated with azathioprine. DESIGN: Prospective, longitudinal study. Laboratory tests and clinical evaluations were performed monthly for 6 months and then every 2 to 3 months (median follow-up, 13.4 months). SETTING: Tertiary care medical center. PATIENTS: Twenty-seven patients with immunobullous disease treated with azathioprine were enrolled during a 2-year period. Twelve met the criteria for evaluation of optimal levels of 6-thioguanine nucleotide. MAIN OUTCOME MEASURES: Blood levels of 6-thioguanine nucleotide, 6-methylmercaptopurine, and thiopurine methyltransferase by polymerase chain reaction and enzyme activity were measured longitudinally during treatment. RESULTS: The range of 6-thioguanine nucleotide was 48 to 457 pmol/8 x 10(8) red blood cells (RBCs), with an average optimal level of 190.7 pmol/8 x 10(8) RBCs for all patients. The mean optimal levels were 179.4 and 205.6 pmol/8 x 10(8) RBCs for pemphigus and pemphigoid, respectively. Limited disease required less 6-thioguanine, with a mean of 145.3 pmol/8 x 10(8) RBCs. Longitudinal induction of thiopurine methyltransferase activity was observed during treatment. Patients with recalcitrant disease showed higher induction of enzyme activity (with an increase of 9.1 to 23.6 U/mL of RBCs above baseline) than did those with responsive disease. CONCLUSIONS: Optimal levels of 6-thioguanine nucleotide metabolites for disease remission in dermatology patients are 150 to 300 pmol/8 x 10(8) RBCs. High levels of the inactive metabolite 6-methylmercaptopurine and induction of thiopurine methyltransferase are associated with recalcitrant disease.

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.