Perioral Erythema Research Articles

Oral manifestations associated with inflammatory bowel disease and early endoscopic findings in patients with spondyloarthritis

Background and aimsSpondyloarthritis (SpA) is a group of autoinflammatory disorders, of which the primary extra-articular manifestation is inflammatory bowel disease (IBD). The oral cavity being a part of gastrointestinal tract, is significantly compromised in IBD, and in many cases, it is the first site of clinical manifestations of IBD. This study aimed to identify changes in the oral mucosa associated with the onset of IBD and their association with endoscopic/histological findings.Materials and methodsThe study assessed 80 patients with SpA and 52 healthy controls. Oral, rheumatological, and gastroenterological assessments were performed. The ileocolonoscopy was performed via digital magnification chromoendoscopy. The statistical analysis consisted of Chi-square, Fisher's exact, and multiple correspondence discriminant analysis tests.ResultsFrom the disease cohort, 63.0% patients showed oral lesions (p = 0.050). These manifestations ranged from gingivitis (55.0%, p = 0.001), aphthous stomatitis (3.8%, p = 0.091), angular cheilitis (2.6%, p = 0.200), and perioral erythema with scaling (1.3%, p = 0.300). All patients who presented with alterations in colonic mucosa also had oral lesions associated with IBD (p = 0.039), specifically gingivitis/aphthous stomatitis (p = 0.029).ConclusionThe patients with SpA without IBD present significant oral signs and symptoms. Gingivitis seems to be the most relevant because of its associations with early endoscopic and histological findings.Clinical relevanceAn integral approach to the diagnostic tests that includes evaluations of oral, rheumatological and gastroenterological tissues may favor timely attention and improve patients’ quality of life.

P050 Oral Inflammatory Changes Associated With Inflammatory Bowel Disease in Spondyloarthritis Associated With Early Endoscopic Findings.

Spondyloarthritis (SpA) is a heterogeneous group of chronic autoinflammatory disorders that can present extra-articular gastrointestinal manifestations. Among them is mainly inflammatory bowel disease (IBD). Although IBD mainly affects the intestinal tract, it can include early manifestations evident in the oral cavity. No comparative data on these oral manifestations in patients with SpA were found in the literature. To identify oral clinical manifestations due to changes in the oral mucosa associated with IBD in patients with SpA without a diagnosis of IBD and associate them with endoscopic and histological findings. 80 patients with SpA and 52 healthy controls were evaluated. They were assessed intra- and extra-orally, following the modified World Health Organization guideline. In addition, by clinical parameters of rheumatological, gastrointestinal and laboratory activity. Ileocolonoscopy was performed with digital chromoendoscopy with magnification and histological analysis. Comparative analyzes were performed by Chi square tests, Fisher's exact tests, confirmed by univariate regression and discriminant analysis of multiple correspondences. Institutional ethics committee approval cod-2017-023. The patients with SpA had 56% male gender, mean age of 42.8 years (SD ± 10.4) and a BMI in the range of 23.9 - 28.4. The healthy controls, 54% of the male gender with an average age of 41 years (SD ± 13.6) and a body mass index-BMI in the range of 22.9 - 27.6. The patients reported smoking only in 6.2%, however as a smoking history in 31% and passive smokers (15%), the majority employed (41%), married (56%) and professionals (49%). Of the healthy controls, they smoked (15%), with a history of smoking (31%), passive smokers (21%), the majority employed (77%), with their own home (67%), and professionals (54%). The patients with SpA reported a greater presence of some signs and symptoms of gastrointestinal origin 69%, while in the controls it was 7.7% (p = 0.001). Forty one of them were referred to colonoscopy with magnification being in 17.1 % changes in the mucosa of the rectum and in the same frequency changes in the mucosa of the sigmoid colon. Regarding the ileum, changes in the mucosa were evidenced in 41.5% of the cases. The presence of oral lesions was evident and predominated in them (63%) compared to controls p = 0.050. The main oral lesions associated with IBD were gingivitis (55%) (p = 0.001), followed by aphthous stomatitis (3.8%), angular cheilitis (2.6%) and perioral erythema with scaling (1.3%). 100% of the patients who presented alteration of the colonic mucosa presented oral lesions associated with IBD (p = 0039), which was also significantly associated with the presence of gingivitis/aphthous stomatitis (p = 0.029). Patients with SpA without a diagnosis of IBD have more oral signs and symptoms compared to healthy controls. Gingivitis is important given its association with early endoscopic and histological findings. Manifestations in the oral cavity can precede intestinal manifestations, therefore the clinical assessment by the oral pathologist in conjunction with gastroenterology and rheumatology allows a timely referral to gastroenterology and an endoscopic and histological evaluation, impacting the quality of life of patients.

MON-587 A Case of Pseudoglucagonoma Syndrome Post Bariatric Surgery

IntroductionNecrotizing migratory erythema (NME) is hallmark clinical finding of Glucagonoma, an islet cell tumor of the pancreas. NME can sometimes be seen in the absence of Glucagonoma, a condition referred to as Pseudo-glucagonoma syndrome(PGS). We report a case of NME associated with severe nutritional deficiency.Case48 y/o female presented to dermatology clinic with diffuse itchy rash of 6 months duration. The rash started on the arms and spread to involve lower abdomen, legs and perioral area. It was unresponsive to both topical and high dose PO steroids. Biopsy of the rash showed confluent parakeratosis and mild spongiosis secondary to nutritional deficiencies vs NME. Her medical history was significant for Nissen fundoplication 20 years ago with a revision to Roux-en Y gastric by-pass, 6 years ago. She did not have a history of Diabetes or Inflammatory Bowel Disease. She was not on any vitamin supplementation, as she did not follow up for post bariatric care. On admission to the hospital for sepsis from secondary infection of the rash, diffuse erythematous, eczematous papules and plaques were noted on bilateral forearms, thighs, calves, buttocks and ankles. She also had perioral erythema and fissuring. Laboratory evaluation showed low prealbumin and multiple nutritional deficiencies including copper, zinc, vitamin B6, Vitamin D, ferritin. Her HbA1c was 5.8 % and fasting glucagon levels were normal. Her liver function was initially normal but later developed transaminitis from septic shock. She was started on enteral feeds with nutritional supplementation. This resulted in significant improvement of her rash with correction of underlying nutritional deficiencies.DiscussionNME in the absence of Glucagonoma is extremely rare and is seen in hepatic cirrhosis, malabsorption disorders, inflammatory bowel disease and nutritional deficiencies including zinc deficiency, Pellagra, Kwashiorkor. Though the exact mechanism for NME in these conditions is unclear. It is postulated that unabsorbed nutrients in the gut lumen are potent stimulators of enteroglucagon which in turn mediates the development of NME. The treatment of NME associated with PGS is to correct the underlying cause. Our patient had history of gastric bypass surgery and did not get routine post bariatric care. She presented with multiple nutritional deficiencies which likely caused NME. It is important to recognize that post bariatric surgery, patients are at risk for both macro and micronutrient deficiencies and hence need frequent nutrition assessment, supplementation and monitoring.ReferencesTierney EP, Badger J. Etiology and pathogenesis of necrolytic migratory erythema: review of the literature. MedGenMed 2004

Mal de Meleda : a case successfully treated with acitretin

Mal de Meleda (MdM) is an autosomal recessive form of palmoplantar keratoderma, that is characterized by transgradient keratoderma with associated scleroatrophy, nail changes, pseudoainhum around digits and perioral erythema, without a tendency for spontaneous resolution. Mal de Meleda can lead to severe flexion contractures in some patients as well as mycotic over-rinfections with significant impact on quality of life and daily activity. Mal de Meleda is a rare genetic disorder but no standardized treatment has been established. Treatment options for this disease include topical keratolytic agents, propylene glycol, topical 5-fluorouracil and surgical treatment. In addition, it has been reported that etretinate and acitretin, usually produce improvement. Herein, we present a case of Mal de Meleda with lips involvement which showed significant clinical improvement with acitritin.

Les manifestations orales de la maladie de Crohn

Crohn’s disease (CD) is a chronic inflammatory bowel disease whose oral features remain underrated at clinical examination. The aim of the study was to report forerunner orofacial presentations leading to diagnosis of CD among nine patients of mean age 23 between 2008 and 2015. Linear ulcerative buccal lesions, cobblestoning, mucosal tags and perioral erythema are highly evocative and might precede intestinal signs for several years. Oral biopsies revealed highly suggestive lesions of CD for 45% of patients before any intestinal symptom or digestive investigation. Mean time to diagnosis was 14 months. Immunomodulatory therapy (azathioprine, infliximab, adalimumab) was introduced alone or in association for 89% of patients. Early diagnosis could lead to specific therapy, often necessary to reduce severity of lesions and improve quality of life.

Die oralen Auswirkungen von Morbus Crohn

Crohn’s disease (CD) is a chronic inflammatory bowel disease whose oral features remain underrated at clinical examination. The aim of the study was to report forerunner orofacial presentations leading to diagnosis of CD among nine patients of mean age 23 between 2008 and 2015. Linear ulcerative buccal lesions, cobblestoning, mucosal tags and perioral erythema are highly evocative and might precede intestinal signs for several years. Oral biopsies revealed highly suggestive lesions of CD for 45% of patients before any intestinal symptom or digestive investigation. Mean time to diagnosis was 14 months. Immunomodulatory therapy (azathioprine, infliximab, adalimumab) was introduced alone or in association for 89% of patients. Early diagnosis could lead to specific therapy, often necessary to reduce severity of lesions and improve quality of life.

Oral Pathology unmasking Gastrointestinal Disease

Abstract Different ggastrointestinal disorders, such as Gastroesophageal Reflux Disease (GERD), Celiac Disease (CD) and Cohn’s disease, may manifest with alterations of the oral cavity but are often under and misdiagnosed both by physicians and dentists. GERD can cause dental erosions, which are the main oral manifestation of this disease, or other multiple affections involving both hard and soft tissues such as burning mouth, aphtous oral ulcers, erythema of soft palate and uvula, stomatitis, epithelial atrophy, increased fibroblast number in chorion, xerostomia and drooling. CD may be responsible of recurrent aphthous stomatitis (RAS), dental enamel defects, delayed eruption of teeth, atrophic glossitis and angular chelitis. Crohn's disease can occur with several oral manifestations like indurated tag-like lesions, clobbestoning, mucogingivitis or, less specifically, with RAS, angular cheilitis, reduced salivation, halitosis, dental caries and periodontal involvement, candidiasis, odynophagia, minor salivary gland enlargement, perioral erythema with scaling, recurrent buccal abscesses, glossitis, mucosal discoloration, lichen planus, and metallic dysgeusia. A prompt detection of the oral signs of these systemic diseases allows an early diagnosis and treatment and could prevent related long-term complications.

A novel homozygous mutation disrupting the initiation codon in the SLURP1 gene underlies mal de Meleda in a consanguineous family.

Mal de Meleda (MDM) is a palmoplantar keratoderma (PPK), characterized by hyperkeratosis of the palms and soles, and keratotic skin lesions. Patients with MDM can develop perioral erythema, keratotic and lichenoid plaques over the joints (including the elbows and knees), nail abnormalities, joint contractures and stiffness, brachydactyly, sclerodactyly, pseudoainhum, and malodorous maceration. MDM is associated with mutations in the SLURP1 gene. We report a consanguineous family in which MDM was inherited in an autosomal recessive manner. Genotyping using microsatellite markers established linkage in the family to the SLURP1 gene, which has been mapped previously to chromosome 8q24.3. Sequence analysis revealed a homozygous missense mutation (c.2T>C, p.Met1Thr) in affected family members. Molecular docking studies using a ZDOCK server predicted disruption of binding of the mutant variant to its target α7-nAChR. This study further supports the previously reported findings that homozygous mutations in the SLURP1 gene cause MDM.

Mal de Meleda with lip involvement: A report of two cases

Mal de Meleda is a rare autosomal recessive transgradient palmoplantar keratoderma characterized by transgradient keratoderma with associated scleroatrophy, nail changes, pseudoainhum around digits and perioral erythema, without a tendency for spontaneous resolution. Involvement of the lip by keratoderma has not been reported in the English literature. Here we present two cases of Mal de Meleda with unusual lip involvement. The first case was a 15-year-old girl, born of second-degree consanguineous marriage, who presented with transgradient palmoplantar keratoderma from 6 months of age, with lichenoid papules and plaques on the elbows and knees, conical tapering of the distal digits, flexion deformity of several fingers, digital constriction, knuckle pads and lip involvement. The second case was a 24-year-old male with transgradient palmoplantar keratoderma since birth. He also had scaly plaques on the extensors of bilateral knees and elbows, knuckle pads, pseudosclerodermatous fingers with conical tapering, digital constrictions at various places with mild flexion deformity and lip involvement. Both patients were otherwise normal without any family history.

Characteristics of patients with orofacial granulomatosis

Orofacial granulomatosis has mostly been described in reports of very small numbers of cases. Few large case groups have been described. The aim of this study was to describe the demographics, symptoms, clinical features and laboratory findings in a large cohort of cases. Clinical and laboratory data for 119 cases of orofacial granulomatosis who attended oral medicine clinics in Dublin, Ireland, were examined for demographic characteristics at the time of first presentation. The male/female ratio was approximately 1:1, with a median age (and range) of 28 (5-84) years. Symptoms had been present for a median duration of 12 weeks. A food association was suspected by 30% of patients. The predominant complaint was lip swelling (77%) with only 15% reporting facial swelling, while 8% complained of both. Almost all patients had clinical evidence of lip or facial swelling (95%). Other common extra-oral manifestations were lip fissuring (30%), angular cheilitis (28%) and perioral erythema (28%). Common intra-oral manifestations were cobblestoning of the buccal mucosa (63%), ulcers (36%), granulomatous gingivitis (33%), mucosal tags (29%) and fissured tongue (17%). Over half of the biopsies (56%) performed were reported as typical of orofacial granulomatosis. This is one of the largest cohorts of orofacial granulomatosis patients to have been described in detail.

Glove-and-stocking-like keratoderma with hyperhidrosis and perioral erythema

Conflict of interest: there are no conflicts of interest. A 28‐year–old Tunisian man presented with extensive palmoplantar hyperkeratosis, which had appeared within the first year of life. Erythema of the palms and soles was the initial manifestation, followed by progressive thickening of the skin. The brother, sister, and relatives in his mother's family had similar findings. There was no known consanguinity in the patient's family. Physical examination revealed disfiguring palmoplantar keratoderma with involvement of the back of the hand and feet, significantly reducing mobility of the hands (Fig. 1). In addition, pachyonychia and palmoplantar hyperhidrosis were present. The patient also had perioral erythema with angular cheilitis. He was otherwise in good general health. ... ... Histopathological examination of a punch biopsy specimen from the dorsum of the right hand stained with haematoxylin and eosin revealed a markedly thickened corneal layer, compact orthokeratosis with focal parakeratosis, an acantholytic broadened surface epithelium with a prominent stratum granulosum, and psoriasiform elongated rete ridges. The upper dermis showed perivascular inflammatory infiltrates predominantly consisting of lymphohistiocytic cells (Fig. 2).

SLURP1 Is a Late Marker of Epidermal Differentiation and Is Absent in Mal de Meleda

SLURP1 is a secreted member of the LY6/PLAUR protein family. Mutations in the SLURP1 gene are the cause of Mal de Meleda (MDM), a rare autosomal recessive genetic disease, characterized by inflammatory palmoplantar keratoderma. In this study, we have analyzed the expression of SLURP1 in normal and MDM skin. SLURP1 was found to be a marker of late differentiation, predominantly expressed in the granular layer of skin, notably the acrosyringium. Moreover, SLURP1 was also identified in several biological fluids such as sweat, saliva, tears, and urine from normal volunteers. In palmoplantar sections from MDM patients, as well as in their sweat, mutant SLURP1, including the new variant R71H-SLURP1, was either absent or barely detectable. Transfected human embryonic kidney 293T cells expressed the MDM mutant SLURP1 containing the single amino-acid substitution G86R but did not tolerate the MDM mutation W15R located in the signal peptide. Thus, most MDM mutations in SLURP1 affect either the expression, integrity, or stability of the protein, suggesting that a simple immunologic test could be used as a rapid screening procedure.

Clinical manifestations of cow milk protein intolerance in infants

The disorder of cow milk protein intolerance is characterised by a wide spectrum of clinical manifestations caused by hypersensitivity of type I, II, or IV, and occurs in 2-3% of children, mostly infants. The aim of this study was to present our experiences and observations of clinical signs and symptoms of cow milk protein intolerance in infants aged below 12 months. The investigation was carried out on a sample of 55 infants, aged between 1.5-9 months (X = 4.21 +/- 1.25), who had cow milk protein intolerance. Diagnosis of illness was based on characteristic anamnestic, clinical, and laboratory parameters, as well as on an adequate patient's response to antigen elimination. The clinical presentation of cow milk protein intolerance was dominated by cutaneous problems, found in 40/55 (72.73%) infants, followed by digestive disorders, found in 31 (56.36%), while respiratory tract disorders were observed least frequently (14.55%). None of the patients developed anaphylactic shock. Changes involving only one system were found in 35 (63.64%) patients; of these 20 (36.36%) were cutaneous and 15 (27.27%) digestive. Twenty (36.36%) infants displayed multisystemic changes; of these 12 were cutaneus with digestive, 4 were cutaneus with respiratory, while 4 infants had cutaneous, digestive, and respiratory disorders. Of the 55 infants with cow milk protein intolerance, 26 (47.27%) had urticaria, 22 (40.00%) perioral erythema, 21 (38.18%) diarrhoea (15 haemorrhagic, 6 non-haemorrhagic), 13 (23.64%) vomiting, 12 (21.82%) Quincke's oedema, 12 (21.82%) eczema, 5 (9.09%) obstructive bronchitis, while 3 (5.45%) infants had laryngitis. In 5 (9.09%) patients we found a significant body weight deficit and in 3 (5.45%), sideropenic anaemia, while longitudinal growth retardation was not registered in any of the patients. Our study showed that the disorder of cow milk protein intolerance predominantly involved cutaneous disorders as well as combinations of cutaneous and digestive disorders, while respiratory system disorders proved to be relatively rare.

Mutations in the gene encoding SLURP-1 in Mal de Meleda.

Mal de Meleda (MDM) is a rare autosomal recessive skin disorder, characterized by transgressive palmoplantar keratoderma (PPK), keratotic skin lesions, perioral erythema, brachydactyly and nail abnormalities. We report the refinement of our previously described interval of MDM on chromosome 8qter, and the identification of mutations in affected individuals in the ARS (component B) gene, encoding a protein named SLURP-1, for secreted Ly-6/uPAR related protein 1. This protein is a member of the Ly-6/uPAR superfamily, in which most members have been localized in a cluster on chromosome 8q24.3. The amino acid composition of SLURP-1 is homologous to that of toxins such as frog cytotoxin and snake venom neurotoxins and cardiotoxins. Three different homozygous mutations (a deletion, a nonsense and a splice site mutation) were detected in 19 families of Algerian and Croatian origin, suggesting founder effects. Moreover, one of the common haplotypes presenting the same mutation was shared by families from both populations. Secreted and receptor proteins of the Ly-6/uPAR superfamily have been implicated in transmembrane signal transduction, cell activation and cell adhesion. This is the first instance of a secreted protein being involved in a PPK.

Clinical and genetic studies of 3 large, consanguineous, Algerian families with Mal de Meleda.

Mal de Meleda (MIM 248300), also referred to as keratosis palmoplantaris transgrediens of Siemens, is a rare autosomal recessive skin disorder with a prevalence in the general population of 1 in 100,000. The main clinical characteristics are transgressive palmoplantar keratoderma, hyperhidrosis, and perioral erythema, but there are also associated features such as brachydactyly, nail abnormalities, and lichenoid plaques. We studied the clinical and genetic characteristics of 3 large, consanguineous, Algerian families, including 14 affected individuals. Homozygosity mapping of the third family confirmed localization of the responsible gene to 8qter in all 3 families. Although some differences in phenotypic expression among subjects were noted, genetic analysis of the 3 families who shared a common ethnic background indicated that a single gene is responsible for mal de Meleda in this population.

Genetic linkage of Meleda disease to chromosome 8qter.

Meleda disease (mal de Meleda) MIM *248300 is an autosomal recessive disorder, clinically characterised by transgressive palmoplantar keratoderma, hyperhidrosis and perioral erythema. It was first described on the Adriatic island of Meleda, where it was relatively common. The prevalence in the general population is estimated to be 1 in 100,000. Linkage analysis of two large consanguineous families from Algeria, including 10 affected individuals, showed strong evidence for localisation of Meleda disease to chromosome 8qter with a maximum two-point lod score for D8S1751 of 8.21 at theta = 0. Analysis of homozygosity regions and recombination events places the gene in a region of at least 3 cM, telomeric to D8S1727. A common haplotype was observed in the two families, suggesting a founder effect.

Allergenicity of milk protein hydrolysate formulae in children with cow's milk allergy.

Cow's milk protein hydrolysate formulae have been developed to lower or eliminate the allergenicity of cow's milk proteins, and to reduce the antigenic load and the risk of sensitization. Cross-reactivity between different hydrolysate formulae and cow's milk proteins has been demonstrated. We have studied 20 children (median age 31 months, range 15-76 months) with a history of IgE-mediated cow's milk allergy. All the children had immediate allergic respiratory and/or cutaneous and/or gastro-intestinal reactions to cow's milk ingestion. In addition, the children had positive prick skin tests and positive RAST to cow's milk. Prick skin test, RAST, and double-blind placebo controlled food challenges were performed with three different hydrolysate formulae: a casein hydrolysate formula and two whey formulae, one partially and one extensively hydrolyzed. All 20 children had immediate allergic reactions after the challenge test with cow's milk. Only 2/20 children had a positive challenge test with a casein hydrolysate formula (Alimentum): one developed asthma and one urticaria. Two of the 15 children challenged with an extensively hydrolysed whey formula (Profylac) developed perioral erythema. Nine out of 20 children had a positive challenge test with a partially hydrolysed whey formula (Nidina H.A.): four developed asthma, three urticaria and two lip oedema. All children had positive prick skin tests to cow's milk proteins (casein and/or lactalbumin); 9 to Nidina H.A.; 3 to Profylac, and 3 to Alimentum. Specific IgE antibodies to cow's milk were present in all children; in 13 to Nidina H.A., in 4 to Profylac, and in 3 to Alimentum.

Oral Crohn's disease and pyostomatitis vegetans: An unusual association

Oral features of Crohn's disease include ulcers, lip fissuring, cobblestone plaques, angular cheilitis, polypoid lesions, and perioral erythema. Pyostomatitis vegetans is a rare eruption of the oral mucosa characterized by tiny yellow pustules. It is considered a marker for inflammatory bowel disease. We describe a 45-year-old woman with a 6-month history of painful sores in her mouth, diarrhea, weight loss, and cutaneous lesions. Oral examination revealed cobblestone plaques and indentation on the tongue and friable vegetating pustules on the labial commissures. Staphylococcus simulans was isolated from the pustules. Laboratory studies revealed leucocytosis, eosinophilia, and low hemoglobin and zinc levels. Histologic study of the labial lesions revealed hyperplastic epithelium with intraepithelial clefts that contain eosinophils and neutrophils. Tongue lesions showed chronic inflammation with noncaseating granulomas. Later, colonoscopy and biopsy demonstrated Crohn's disease of the anorectal region. Pyostomatitis vegetans lesions regressed after oral zinc supplementation. Prednisone treatment resulted in healing of the tongue lesions. In our patient, pyostomatitis vegetans appeared to be related to zinc deficiency that may have been caused by malabsorption. The pathogenetic interrelationship between pyostomatitis vegetans and Crohn's disease is discussed.

Circumoral dermatitis and cheilitis caused by tartar control dentifrices

Twenty women had similar symptoms and clinical findings related to the use of tartar control toothpaste. Each had burning and itching around the corners of the mouth, followed by pruritic perioral patches of erythema 4 to 14 days later. AU patients had begun using tartar control toothpaste 1 to 2 weeks before onset and had brushed their teeth at least three times per day. The typical findings included a moderately severe perioral erythema with Assuring of the angles of the mouth; scaling erythematous patches, separated by normal skin lateral to the initial erythema, were also present. No vesiculation or crusting was noted, and involvement of the gingiva and buccal mucosa was noted in only one case. On discontinuation of the tartar control toothpaste, marked improvement was seen within 1 to 6 weeks with, in most instances, either no treatment or 1% hydrocortisone cream.

Ginkgo-Tree Dermatitis, Stomatitis, and Proctitis

ALLERGENS causing skin sensitization also can result in allergic contact reactions of mucous membranes including cheilitis, stomatitis, proctitis, and pruritus ani. The present case illustrates an unusual cause of such allergy from the fruit of the female ginkgo tree. Report of a Case A 63-year-old man was observed in the Dermatology Clinic for porphyria cutaneatarda. His urinary uroporphyrins had decreased to 227μg/24 hr with abstinence from alcohol. During a scheduled return visit, perioral erythema and swelling were noted. The patient related a history of burning, tingling, and soreness of his mouth, and a thick feeling in his tongue which began that morning. He denied the ingestion of new foods, new toothpaste, chewing gum, or oral medications. Results of a physical examination showed perioral erythema, swollen lips, and erythema of the buccal mucosa, tongue, and tonsillar pillars. The patient was advised to change toothpaste, avoid chewing gum, gargle with salt water,