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.     

Oral mucosal diseases: erythema multiforme

Erythema multiforme (EM) is a rare acute mucocutaneous condition caused by a hypersensitivity reaction with the appearance of cytotoxic T lymphocytes in the epithelium that induce apoptosis in keratinocytes, which leads to satellite cell necrosis. EM can be triggered by a range of factors, but the best documented association is with preceding infection with herpes simplex virus (HSV). Most other cases are initiated by drugs. EM has been classified into a number of variants, mainly minor and major forms, as it may involve the mouth alone, or present as a skin eruption with or without oral or other lesions of the mucous membrane. EM minor typically affects only one mucosa, and may be associated with symmetrical target skin lesions on the extremities. EM major typically involves two or more mucous membranes with more variable skin involvement. A severe variant of EM major is Stevens-Johnson syndrome, which typically extensively involves the skin. Both EM major and Stevens-Johnson syndrome can involve internal organs and produce systemic symptoms. Treatment of EM is controversial, as there is no reliable evidence. Precipitants should be avoided or treated and, in severe cases, corticosteroids may be needed. Toxic epidermal necrolysis may be similar to Stevens-Johnson syndrome, but many experts regard it as a discrete disease, and therefore it is not discussed here.     

Erythema multiforme and related disorders

Erythema multiforme (EM) and related disorders comprise a group of mucocutaneous disorders characterized by variable degrees of mucosal and cutaneous blistering and ulceration that occasionally can give rise to systemic upset and possibly compromise life. The clinical classification of these disorders has often been variable, thus making definitive diagnosis sometimes difficult. Despite being often caused by, or at least associated with, infection or drug therapy, the pathogenic mechanisms of these disorders remain unclear, and as a consequence, there are no evidence-based, reliably effective therapies. The present article reviews aspects of EM and related disorders of relevance to oral medicine clinical practice and highlights the associated potential etiologic agents, pathogenic mechanisms and therapies.     

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.     

Pemphigus vulgaris in adolescence: case report

Pemphigus vulgaris is an uncommon mucocutaneous disease caused by autoantibodies against desmosomal antigens. It affects mainly middle-aged adults, and juvenile cases are rare. The authors present a case of pemphigus vulgaris in adolescence and review the literature. A 16-year-old girl showed oral and cutaneous lesions suggestive of pemphigus vulgaris. Histopathology and direct immunofluorescence of the oral mucosa confirmed the diagnosis and systemic steroid therapy was efficient in controlling the disease. The recognition of the oral lesions of pemphigus by the clinician, its early diagnosis, and prompt therapy are essential for a favorable prognosis.     

Herpes Simplex Virus Type 1 infection: overview on relevant clinico‐pathological features*

Herpes Simplex Virus Type 1 (HSV‐1) is a nuclear replicating enveloped virus, usually acquired through direct contact with infected lesions or body fluids (typically saliva). The prevalence of HSV‐1 infection increases progressively from childhood, the seroprevalence being inversely related to socioeconomic background. Primary HSV‐1 infections in children are either asymptomatic or following an incubation period of about 1 week gives rise to mucocutaneous vesicular eruptions. Herpetic gingivostomatitis typically affects the tongue, lips, gingival, buccal mucosa and the hard and soft palate. Most primary oro‐facial HSV infection is caused by HSV‐1, infection by HSV‐2 is increasingly common. Recurrent infections, which occur at variable intervals, typically give rise to vesiculo‐ulcerative lesions at mucocutaneous junctions particularly the lips (herpes labialis). Recurrent HSV‐1 infection within the mouth is uncommon in otherwise healthy patients, although in immunocompromised patients, recurrent infection can be more extensive and/or aggressive. The diagnosis of common herpetic infection can usually be based upon the clinical history and presenting features. Confirmatory laboratory diagnosis is, however, required when patients are, or may be, immunocompromised.     

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.     

Chronic indolent orofacial herpes simplex virus infection in chronic leukemia: a report of three cases

Orofacial mucocutaneous infections caused by herpes simplex virus (HSV) may exhibit a distinct chronic indolent pattern of behavior in some immunosuppressed patients as opposed to the more familiar aggressive patterns. Three patients with chronic leukemia who illustrate this chronic indolent pattern are presented. These cases should alert the clinician to the variable clinical appearance that HSV may adopt in the immunosuppressed patient.     

Linear IgA dermatosis induced by a new angiotensin-converting enzyme inhibitor

A 68-year-old female patient treated with benazepril for arterial hypertension developed oral and cutaneous blistering. Biopsy of the oral and cutaneous lesions showed neutrophilic microabscesses in the mesenchymal papillae, with epitheliomesenchymal separation. Direct immunofluorescence revealed linear immunoglobulin deposits at the epithelial basement membrane zone, consisting predominantly of IgA. The histologic results supported the clinical diagnosis of drug-induced linear IgA disease. The substitution of benazepril with a beta blocker resulted in complete resolution of all mucocutaneous lesions.     

Localized Leishmaniasis of the oral mucosa. A report of three cases

The term leishmaniasis comprises a group of diseases caused by different species of a protozoon called Leishmania. Leishmaniasis is found worldwide, and is considered to be endemic in 88 countries. There are three main clinical forms of leishmaniasis: visceral leishmaniasis, cutaneous leishmaniasis and mucocutaneous leishmaniasis. Exclusive involvement of the mucosa is very rare. We present a series of three cases of mucosal leishmaniasis located in the oral cavity. The fact that all three cases were recorded in Spain (an area where L. infantum is endemic), suggests that the latter was the causal agent. The only manifestation of leishmaniasis disease in the described cases was the appearance of an oral lesion. Treatment was provided in the form of meglumine antimoniate in two patients, with a favorable response. One of the patients left the hospital after diagnosis, without receiving treatment, and the subsequent course is not known. A review is made of the literature on the subject.     

Herpes zoster of the trigeminal nerve: a case report and review of the literature

Herpes zoster (shingles) is caused when the varicella zoster virus that has remained latent since an earlier varicella infection (chicken-pox) is reactivated. Herpes Zoster is a less common and endemic disease than varicella: factors causing reactivation are still not well known, but it occurs in older and/or immunocompromised individuals. Following reactivation, centrifugal migration of herpes zoster virus (HZV) occurs along sensory nerves to produce a characteristic painful cutaneous or mucocutaneous vesicular eruption that is generally limited to the single affected dermatome. Herpes zoster may affect any sensory ganglia and its cutaneous nerve: the most common sites affected are thoracic dermatomes (56%), followed by cranial nerves (13%) and lumbar (13%), cervical (11%) and sacral nerves (4%). Among cranial nerves, the trigeminal and facial nerves are the most affected due to reactivation of HZV latent in gasserian and geniculated ganglia. The 1st division of the trigeminal nerve is commonly affected, whereas the 2nd and the 3rd are rarely involved. During the prodromal stage, the only presenting symptom may be odontalgia, which may prove to be a diagnostic challenge for the dentist, since many diseases can cause orofacial pain, and the diagnosis must be established before final treatment. A literature review of herpes zoster of the trigeminal nerve is presented and the clinical presentation, differential diagnosis and treatment modalities are underlined. A case report is presented.     

Erythema multiforme and herpes simplex virus

It has been suggested that herpes simplex virus (HSV) can trigger erythema multiforme (EM) at different times. One recent study showed HSV antigen in immune complexes of patients with EM. The purpose of our study was to assess a possible association between EM and HSV. Sixteen patients and 16 matched controls were studied using an enzyme-linked immunosorbent assay (ELISA) to measure antibody of the IgG, IgA, and IgM classes against HSV-1. From our study on patients with oral erythema multiforme, we found no evidence to correlate the occurrence and/or severity of EM and the HSV-1.     

Lichen planus. Clinical diagnosis, epidemiology and pathogenesis

Lichen planus is a common mucocutaneous disease of unknown etiology which was initially described a century ago. The specific etiology remains unclear. A number of relationships between lichen planus and other conditions have been noted. The lesions occur most commonly on the buccal mucosa. Gingival involvement occurs in approximately 10% of reported cases. The appearance of lichen planus is extremely variable which leads to confusion with other clinically similar disease entities.     

Cowden syndrome: mucocutaneous lesions as precursors of internal malignancy

Background

Cowden syndrome is an autosomal-dominant hereditary cancer syndrome with high variability and susceptibility. It is characterized by multiple hamartomas and neoplasms of ectodermal, endodermal and mesodermal origin affecting many organs and also by the increased risk of development of internal malignancies.

Case report

A 62-year-old woman was referred to our Maxillofacial Unit with a hamartomatous mass of the left lateral tongue which had slowly grown and was obstructing normal speech and restricting oral intake. The patient had a known history of Cowden syndrome and underwent excision of the lesion under general anaesthetic.

Discussion

Orofacial mucocutaneous features are very common in multiple hamartoma and neoplasia syndrome with almost up to 90 % of the patients being affected. These cutaneous and mucosal lesions, which are predominantly benign, often manifest prior to the development of the internal malignant tumours associated with the syndrome.

Conclusions

The prompt identification of Cowden syndrome’s plethoric signs and symptoms can lead to appropriate surveillance and multidisciplinary management. Oral manifestations are frequent, prominent and usually precede the establishment of malignant tumours of visceral organs; hence, the maxillofacial surgeon or general dentist may have a significant role in the recognition of the disease. Overall prognosis is dependent on prevention or early treatment of internal malignancies; consequently, early diagnosis together with frequent follow-up forms the cornerstone of management.     

Oral lesions in patients with psoriasis: clinical presentation and management

Psoriasis is a chronic inflammatory skin disease that rarely involves the oral cavity. In this report we describe 2 cases, initially diagnosed with cutaneous psoriasis, that present with oral lesions on the attached gingiva. The clinical appearance and differential diagnosis are presented and discussed. Case 1 describes the non-surgical management of intraoral psoriasiform lesions and the use of a free gingival graft to restore an area of gingival recession resulting from an oral lesion. The second case outlines the use of topical corticosteroid therapy as an adjunct to non-surgical periodontal therapy. Although patients with cutaneous psoriasis rarely present with oral involvement, the clinician should be aware that oral lesions may occur. Accurate diagnosis is dependent on a thorough clinical examination, a biopsy of the oral lesions, and a history of cutaneous psoriasis.     

A long-term prospective clinical study of orofacial herpes simplex virus infection in acute leukemia

Orofacial mucocutaneous infections resulting from herpes simplex virus (HSV) were detected in 40% of patients with acute leukemia. Of the 34 separate episodes, oral mucosal sites were involved in 22 cases. Evidence to support dissemination of HSV was found in 3 patients on 4 separate occasions. The relationship of neutrophil levels to the onset and resolution of lesions is examined. The value of acyclovir for treatment of these HSV-induced lesions is reported, and the question of administering this agent for routine prophylaxis against HSV in these patients is addressed.     

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.     

The importance of multidisciplinary healthcare for paraneoplastic pemphigus

Paraneoplastic pemphigus (PNP) is a mucocutaneous disease that occurs in association with an underlying neoplasm. Oral mucosal lesions may be the only manifestation of this condition, or they may be observed in association with cutaneous lesions. The prognosis of PNP is generally poor, and the disease is often fatal. This article highlights an aggressive case of PNP that initially presented with oral mucosal lesions and emphasizes the importance of a multidisciplinary approach for evaluation and management of this condition.     

Oral manifestations of patients with lupus erythematosus

Lupus erythematosus manifests as cutaneous variants, such as discoid lupus erythematosus or systemic lupus erythematosus. Systemic lupus erythematosus is a multisystem autoimmune disease characterized by general autoantibody production and a wide range of mucocutaneous, renal, neuropsychiatric, cardiovascular, infectious, and hematologic manifestations. This article discusses the prevalence of and considerations for oral mucosal lesions in lupus erythematosus and the impact of the various disease manifestations of systemic lupus erythematosus on dental management.     

Factitious Injury of the Oral Mucosa: A Case Series

Gingival and oral mucosal tissues can be the site of a number of mucocutaneous and ulcerative conditions. Generally, these are not difficult to identify on the basis of clinical characteristics, and diagnosis can be aided by the use of routine histopathological and immunopathological techniques as well as other laboratory investigations. Self-induced or factitious injury (FI) of the oral mucosal tissues may present a confusing clinical picture, and be diagnosed erroneously as a mucocutaneous disorder in spite of the absence of appropriate pathological and immunopathological findings, or a failure to respond to routine treatment. A case series is presented here outlining 4 cases of FI which presented initially as mucocutaneous disease. These cases were investigated to rule out systemic or local causes, in order to establish a diagnosis of FT Treatment of these conditions was facilitated with placebo or sham procedures which were designed primarily to cover the lesions. In most cases, the self injurious behavior could be linked to secondary gain. J Periodontol 1995;66:241–245.