Mucosal disease series. Number IV. Erythema multiforme

Erythema multiforme (EM) is an acute mucocutaneous hypersensitivity reaction characterised by a skin eruption, with or without oral or other mucous membrane lesions. Occasionally EM may involve the mouth alone. EM has been classified into a number of different variants based on the degree of mucosal involvement and the nature and distribution of the skin lesions. EM minor typically affects no more than one mucosa, is the most common form and may be associated with symmetrical target lesions on the extremities. EM major is more severe, typically involving two or more mucous membranes with more variable skin involvement - which is used to distinguish it from Stevens-Johnson syndrome (SJS), where there is extensive skin involvement and significant morbidity and a mortality rate of 5-15%. Both EM major and SJS can involve internal organs and typically are associated with systemic symptoms. Toxic epidermal necrolysis (TEN) may be a severe manifestation of EM, but some experts regard it as a discrete disease. EM can be triggered by a number of factors, but the best documented is preceding infection with herpes simplex virus (HSV), the lesions resulting from a cell mediated immune reaction triggered by HSV-DNA. SJS and TEN are usually initiated by drugs, and the tissue damage is mediated by soluble factors including Fas and FasL.     

Stevens-Johnson syndrome: case presentation

Erythema Multiforme (EM) is a rare mucocutaneous disease with a variety of clinical manifestations. EM it was recognized in the early 1800's, and still the etiology is unknown. It has been recently suggested erythema multiforme (EM) major and Stevens-Johnson Syndrome (SJS) could be separated as two distinct clinical disorders with similar mucosal erosions, but different patterns of cutaneous lesions. In particular SJS should be used for a syndrome characterized by mucous membrane erosions and widespread small blisters that appear on erythematous or purpuric maculae, which are different from classic targets. In SJS mouth, eyes, skin, genitalia and occasionally the esophagus and respiratory track may be affected. Oral lesions may cause severe pain and usually lips may become encrusted. Concerning ocular involvement, if there is conjunctivitis or uveitis this may lead to scarring and blindness. Also, the course of disease and the prognosis are in most cases severe.     

Oral erythema multiforme major: a triple case report

This article presents three cases of erythema multiforme (EM) major with predominant oral mucosal lesions. Two males, aged 28 and 10, and a 19-year-old woman came to the clinic with oral vesiculoerosive lesions consistent with a diagnosis of EM major. All three patients reported histories consistent with an initial oral herpetic infection. Management with topical and systemic steroids resolved the lesions successfully in all three cases. The relatively recent literature has documented differences between EM and Stevens-Johnson syndrome. It is important for dentists to understand the etiology of EM and the associated diagnostic and management issues.     

SAPHO syndrome with TMJ involvement: review of the literature and case presentation

Synovitis, acne, pustulosis, hyperostosis, osteitis (SAPHO) syndrome is a rare group of sterile, inflammatory osteoarticular disorders classically associated with skin manifestations. The etiology is unknown but probably involves genetic, infectious, and immunological components. The characteristic feature of the disease is found in the bone lesions, which typically involve the anterior chest wall and axial skeleton. In the literature review, six case reports discussed involvement of the TMJ. Treatment of SAPHO is geared toward symptom management as there is no cure. Surgery for mandibular lesions is usually a last resort as results are reported to be temporary with symptoms recurring within a year. Surgery appears to be performed early after diagnosis of TMJ related pathology; probably because lesions affecting the TMJ involve some limitation in mouth opening with varying degrees of ankylosis. The authors provide a literature review and describe a case of SAPHO syndrome with ankylosis of the left TMJ. The patient was treated with joint reconstruction using a patient-fitted total joint prosthesis (TMJ Concepts Inc., Ventura CA) in single stage surgery. This paper is the first to report maxillary involvement in SAPHO syndrome.     

Herbal medicine induced Stevens-Johnson syndrome: a case report

A severe form of Erythema multiforme (EM), diagnosed as Stevens-Johnson syndrome (SJS) was detected in a 10-year-old Indian male child who took herbal medication of plant origin prescribed by a traditional healer for remission of his fever. The child developed a severe and florid form of mucocutaneous oral ulceration, severe ocular manifestations, along with marked constitutional disturbances and dehydration. An extensive literature search revealed few previous reports of herbal drug induced EM and SJS. In India, traditional therapy with herbal preparation (Ayurvedic medicine) is not uncommon.     

Update on mucous membrane pemphigoid: a heterogeneous immune-mediated subepithelial blistering entity.

Most oral involvement in the skin diseases (dermatoses) is related to mucous membrane pemphigoid or lichen planus. Mucous membrane pemphigoid was the subject of a European Symposium held in Turin, Italy, in June 1997. This review is based on that symposium. Mucous membrane pemphigoid is a subepithelial vesiculobullous disorder mainly of late middle age; it has a slight predilection for women. Whereas mucous membrane pemphigoid was formerly considered a single entity, it is now quite evident that a number of subepithelial vesiculobullous disorders may produce similar clinical pictures and also that a range of variants of mucous membrane pemphigoid exists, with antibodies directed against various hemidesmosomal components or components of the epithelial basement membrane. The term immune-mediated subepithelial blistering diseases has therefore been used. Diagnosis and management of immune-mediated subepithelial blistering diseases on clinical grounds alone are impossible; a full history, general and oral examinations, and biopsy with immunostaining are now invariably required, sometimes supplemented with other investigations. Most patients with mucous membrane pemphigoid affecting the mouth manifest desquamative gingivitis, a fairly common complaint typically seen in women who are middle-aged or older. Oral vesicles and erosions may also occur, and there can be a positive Nikolsky sign. Some patients have lesions of other stratified squamous epithelia, presenting as conjunctival, nasal, oesophageal, laryngeal, vulval, penile, or anal involvement. Apart from improving oral hygiene, immunomodulatory-in particular, immunosuppressive-therapy is typically required to control oral lesions in mucous membrane pemphigoid. No single treatment regimen reliably controls all these disorders.     

Psoriasis of the dorsal surface of the tongue

Psoriasis is primarily an inherited inflammatory skin disease, it is characterized by erythemato-squamous lesions that usually involve elbows, knees and the scalp. Oral manifestations are rare in psoriasis, infact, oral psoriasis involves 2% of psoriatic patients and usually it is observed with the onset of cutaneous lesions and progresses with them. Differential diagnosis should be done for Reiter's syndrome, leukoplakia and geographic tongue. The authors describe a case of tongue psoriasis without cutaneous lesions.     

Erythema multiforme: a literature review and case report

Erythema multiforme is a florid mucocutaneous disease characterized by oral, cutaneous, and ocular manifestations. The cutaneous lesions are pathognomonic because of their unique "target-like" appearance. A severe form of EM has been termed "Stevens-Johnson Syndrome". Although the etiology of EM is unknown, much of the research suggests an immunological association with HSV. The diagnosis of EM is based on signs and symptoms, and a differential diagnosis should include other ulcerative, mucocutaneous diseases, such as erosive lichen planus, pemphigus, varicella zoster, ANUG, TEN, aphthous stomatitis, and primary HSV. Therapeutic measures are palliative, including a soft bland diet, topical anesthetics, and corticosteroids. A case of EM is described which underscores the appearance of the disease and its clinical course.     

Cell-protective effect of human saliva specific for herpes simplex virus

Saliva from normal human adults pre-incubated with human amnion cell cultures protected these cells against destruction by herpes simplex virus. In two-thirds of subjects tested, protection amounted to plaque reduction of 55–94 per cent. Protection seemed to be achieved by direct action on cells rather than inactivation of virus. Evidence indicates that the active material was not antibody, although it was more active against herpes simplex virus than vaccinia or vesicular stomatitis virus. This cell-protective activity correlated with recurrent herpes labialis and may be significant in the varying resistance of individuals to symptomatic recurrences and may explain the tendency of recurrent lesions to involve the lips and circumoral skin although sparing the oral mucous membrane.     

Erythema multiforme secondary to herpes simplex infection: a case report

BACKGROUND: Erythema multiforme (EM) is a complex disease that may have cutaneous and/or mucosal involvement. The severity may range from mild to severe and potentially life threatening. The literature cites many factors including viruses, infections, and medications as causes. This report documents a patient who developed EM secondary to a herpes simplex viral (HSV) infection. METHODS: Two weeks following an eruption of herpes labialis, a 20-year-old white female patient developed acutely painful oral and labial ulcers accompanied by target skin lesions. A diagnosis of erythema multiforme (EM) was made. The patient was treated with antivirals, analgesics, and symptomatic therapy. RESULTS: Nine days after the onset of symptoms, the oral and cutaneous lesions had started to heal and the patient no longer required pain medication. CONCLUSIONS: Although the etiology of EM is still often unknown, infections with herpes simplex virus have been implicated as a possible precipitating factor. This case illustrates the association of the occurrence of EM with an HSV infection.     

Immunopathological diagnosis of cicatricial pemphigoid with desquamative gingivitis. A case report

Cicatricial pemphigoid (CP) is a chronic subepidermal bullous dermatosis which primarily involves the mucous membranes. The oral cavity and the eye are most frequently involved. Since extension of the lesion into the pharynx and esophagus causes sore throat and dysphagia and progressive ocular lesions may cause blindness, early and valid diagnosis is very important. Here we present a case of cicatricial pemphigoid with onset at age 45 in a patient who manifested severe periodontal disease and showed the lesion on the mucous membranes of the mouth (desquamative gingivitis), skin, and eyes. Since definite diagnosis is very important, we describe how we made a differential diagnosis from other diseases which also accompany desquamative gingivitis. We examined the clinical manifestations, blood test results, HLA-genotype, histopathologic findings of the affected tissue, and immunological findings in relation to autoimmunity. Since many of the CP cases are first referred to periodontists or dentists, we believe that the diagnostic strategy described in the present study will be quite informative for making rapid and definite diagnoses of similar cases.     

Management of patients with ocular manifestations in vesiculobullous disorders affecting the mouth

Pemphigoid and pemphigus diseases as well as Stevens–Johnson syndrome present as vesiculobullous disorders of the skin and may additionally involve both the oral cavity and the ocular surface. Ocular involvement ranges from mild irritation and dry eye disease to chronic conjunctivitis, symblepharon, eyelid malposition, ocular surface scarring and severe visual loss. In addition to diagnostic assessments, ophthalmologists must treat the dry eye and meibomian gland dysfunction components of these diseases using a stepladder approach, including eyelid hygiene and lubricants. Topical anti‐inflammatory therapy is used to treat acute inflammatory exacerbations of the ocular surface, but it cannot prevent scarring alone. Intralesional antimetabolite therapy can cause regression of conjunctival pathology in selected cases. Hence, patients with vesiculobullous disorders should be managed by a multidisciplinary team representing ophthalmology, dermatology, otolaryngology, oral medicine and pathology, internal medicine and intensive care. Systemic treatments including corticosteroids, azathioprine, cyclophosphamide, cyclosporine and mycophenolate mofetil help control inflammation. Intravenous immunoglobulins, plasmapheresis and targeted antibody therapy can be used in selected, severe and treatment‐resistant cases. Local surgical management may include debridement of pseudomembranes, lysis of symblepharon, amniotic and mucous membrane grafting as well as reconstructive procedures. Prospective, multicentre, international studies are recommended to further support evidence‐based practice.     

Cowden's syndrome: a case report.

Cowden's syndrome, a rare genodermatosis of autosomal-dominant inheritance with variable expressivity, is characterized by a combination of ectodermal, mesodermal, and endodermal hamartomas that may involve the skin, mucous membranes, breasts, gastrointestinal tract, and thyroid. A 26-year-old woman who presented for replacement of her teeth, all of which had been extracted because of rapidly progressive periodontitis. She was diagnosed with Cowden's syndrome based on mucocutaneous abnormalities, thyroid involvement, and abnormalities of the skeletal and genitourinary systems. The clinical significance and differential diagnosis of this disease are highlighted.     

Calcified subgaleal hematoma with secondary cranial deformity in a patient with Kasabach-Merritt phenomenon

We present here the case of a 25-month-old boy who, in conjunction with his preexisting Kasabach-Merritt phenomenon (KMP), developed a massive calcified subgaleal hematoma (SGH) with secondary cranial deformity. Kasabach-Merritt phenomenon is a rare condition that involves a secondary consumptive coagulopathy in the setting of congenital hemangiomatous lesions. The vascular lesions of KMP are often large, singular, and cutaneous, but may also be diffuse, and involve internal structures. Subgaleal hematomas are a rare condition, most frequently observed in neonates as a complication of instrument-assisted delivery. There have been few cases reported beyond the perinatal period, but those that are present within the literature have typically resulted from some instance of minor trauma to the scalp. Most cases resolve spontaneously and without complication. However, our patient's SGH developed into a large, organized, and calcified lesion, likely with some contribution from his hematologic deficit. In addition, this subgaleal lesion resulted in a cranial deformity, ultimately requiring surgical evacuation and reconstruction. We believe the case presented here represents the first report of an SGH in the setting of KMP.     

Papillary cystadenoma of the lower lip exhibiting ciliated pseudostratified columnar epithelium: report of a bizarre case and review of the literature

Background

Salivary gland tumors are uncommon and constitute 2–6.5 % of all head and neck neoplasms. Tumors of minor salivary gland origin account for less than 25 % of all salivary gland neoplasms. Papillary cystadenoma of salivary glands is a rare benign epithelial neoplasm characterized by multicystic growth in which the epithelium exhibits adenomatous proliferation. Papillary cystadenoma of minor salivary glands most frequently involves the lip, buccal mucosa, and palate. This tumor typically presents as a slow-growing, painless mass, usually with diameter of less than 1 cm and clinical resemblance to a mucocele. Although most papillary cystadenomas are predominantly of one cell type, a regional variability may be present.

Case Report

We present a case of papillary cystadenoma of the minor salivary glands in a 58-year-old patient exhibiting an upper respiratory tract epithelium, a profoundly atypical benign tumor.

Discussion

This type of minor salivary gland tumor epithelium in the lower lip may be the result of a metaplastic process or simply another neoplastic manifestation of papillary cystadenoma. As far as the differential diagnosis of this entity is concerned, it is important to distinguish it from papillary cystadenoma lymphomatosum (Warthin's tumor), low-grade mucoepidermoid carcinoma, the papillary-cystic variant of acinic cell carcinoma, and cystadenocarcinoma Recognition of this lesion is important for the clinician since the differential diagnosis includes lesions with similar clinical appearance and infiltrative behavior.     

Comparison between true and partial hemifacial hypertrophy

Hemifacial hypertrophy (HFH) is rare and characterized by unilateral enlargement of the head and teeth. Hemifacial hypertrophy is classified as true HFH (THFH) with unilateral enlargement of the viscerocranium, and partial HFH (PHFH) in which not all structures are enlarged. We present a case of THFH and compare and contrast it with a case of PHFH. Hemifacial hypertrophy may cover a wide spectrum of defects or may involve only muscle or bone. Myohyperplasia, reported previously as a separate syndrome, may actually represent a forme fruste of PHFH or THFH. The PHFH patient lead a healthy, normal life without significant psychosocial problems and was reluctant to accept his deformity. Contrastingly, the THFH patient had significant social stigma and compromised health together with major aesthetic morbidity. Treatment planning in THFH is arduous and involves multiple modalities. Therefore, it is imperative to differentiate THFH and PHFH for better understanding and management of the condition.     

Fetal alcohol syndrome: report of case

A report of a patient with fetal alcohol syndrome has been presented with a review of the syndrome and the literature. Although the patient was treated in the operating room, many patients with less severe medical involvement or less extensive dental needs can be treated as outpatients. Each case must be considered individually with the prime concern being the patient's safety. Such patients may have numerous emotional or physical problems that the dentist must recognize. These patients are at high risk for several types of heart defects, many of which will require consultation for bacterial endocarditis precautions. The major and minor anomalies associated with fetal alcohol syndrome have been outlined.     

Pemphigus: update for the general practitioner

Pemphigus is a dermatologic disease that can affect both the skin and mucous membranes. Pemphigus affects the oral cavity; the most common form of the disease that is observed clinically is pemphigus vulgaris. Oral lesions may precede skin lesions; therefore, it is imperative that clinicians are aware of the clinical signs and symptoms of this disorder. This article will review the etiology, symptomatology, diagnostic tools, and treatments available to diagnose and manage this disease.     

Dental and anaesthetic management of children with dystrophic epidermolysis bullosa

Background.  Epidermolysis bullosa (EB) is a genetic disease associated with fragility and bullous lesions of the skin and mucous membranes. There are various patterns of inheritance and histopathology. The disease is associated with systemic and oral manifestations, among which may be dental decay necessitating oral rehabilitation. General anaesthesia is frequently required for oral rehabilitation in children with dystrophic EB. Paediatric dentists should be aware of the implications of dystrophic EB for dental and anaesthetic management.
Case report.  Two siblings with moderate to severe systemic and oral manifestations of dystrophic EB requiring extensive oral rehabilitation for rampant decay are described, including special anaesthetic techniques required for airway management and maintenance of skin integrity.
Conclusion.  Dystrophic EB is a rare genetic disorder in which vesiculobullous lesions occur with erosions and scarring of cutaneous and extracutaneous surfaces in the oral cavity. Poor oral hygiene results from efforts to avoid mucosal trauma, resulting in decay. The comprehensive care of children with dystrophic EB impacts not only dental management but anaesthesia administration as well.     

Minor salivary gland calculi. A clinical and histopathological study of 49 cases

The clinical and histopathological characteristics of 49 previously unpublished cases of sialolithiasis of minor salivary glands are presented. The lesions are usually solitary, small, submucosal nodules, which are hard or firm and freely movable in the surrounding tissue and on rare occasions may be multiple. They are most often seen in the buccal mucosa and the upper lip of patients in the 5th, 6th or 7th decade, but are rarely found in the lower lip and palate. The authors found that clinicians usually failed to include calculi in the differential diagnosis and further, they concluded that minor salivary gland calculi are more common than generally believed. In contrast to other studies of calculi in major and minor salivary glands, the periphery of the stones was found to be less frequently lamellated and mineralized. The authors also confirmed the finding of mucous cell metaplasia of ductal epithelium and commented on its possible role in the pathogenesis of sialolithiasis.