Human orf infection complicated by erythema multiforme

A 35-year-old woman developed an orf infection of the fingers after contact with an infected goat. Following the primary orf infection, erythema multiforme developed and was controlled with prednisone. The association between erythema multiforme and orf is discussed; also, it is noted that orf is another viral condition that should be added to the agents that can initiate erythema multiforme.     

Rapid improvement of human orf (ecthyma contagiosum) with topical imiquimod cream: report of four complicated cases

Orf is a zoonosis caused by an epitheliotropic DNA parapox virus. Human orf is a generally benign, self-limiting condition that usually regresses in 6-8 weeks without specific treatment. However, it may be accompanied by local symptoms including pain, pruritus, lymphangitis and axillary adenitis, or less frequently by systemic symptoms such as fever or malaise. Furthermore, it may be complicated by erythema multiforme, Stevens-Johnson syndrome, erysipelas, generalized mucocutaneous eruption, toxic erythema, eyelid oedema and giant, persistent or recurrent lesions in immunocompromised patients. Imiquimod, a potent topical immune response modifier, enhances both the innate and acquired immunity by stimulation of immune system cells resulting in local antiviral, antitumour and immunoregulatory activity. We present, for the first time, four complicated cases of orf successfully treated by topical imiquimod resulting in rapid regression of both orf and associated lesions. Two of the cases were complicated with erythema multiforme, one with recurrent eyelid oedema, and another had giant orf associated with axillary lymphadenitis. We suggest that topical imiquimod may be an effective and safe therapy for complicated orf cases.     

Erythema Multiforme in a 25‐Day Old Neonate

Erythema multiforme is an acute, self‐limiting, mucocutaneous hypersensitivity reaction characterized by distinctive target lesions. Most cases have been attributed to infection. Erythema multiforme occurs mainly in young adults and is extremely rare during the neonatal period. We report a 25‐day‐old girl who presented with target skin lesions on both the palms and soles with no other associated symptoms. She had no remarkable maternal, birth, or past medical history. Complete blood count, urinalysis, chest radiography, and herpes simplex virus 1 and 2 immunoglobulin G (IgG) titers revealed no abnormalities. Pathologic examination showed vacuolar interface change and dyskeratotic cells in the epidermis consistent with erythema multiforme. This unusual case emphasizes the importance of recognizing diagnostic clues in examining patients. Even in the presence of uncharacteristic factors, the typical target lesions of erythema multiforme are distinctive.     

Erythema multiforme‐like eruption localized to a sun‐exposed area

We report on a 35‐year‐old woman with cutaneous lesions characterized by an erythema multiforme‐like appearance localized in the photo‐distributed pattern. She had no history of systemic drug ingestion, herpes simplex virus or any other infection, possible causes of erythema multiforme, before the sun exposure. She had normal tolerance to a phototest, but photoprovocation tests could not be performed because she did not agree to them. This case was diagnosed to be an erythema multiforme‐like variant of a polymorphous light eruption; the differential diagnosis of target‐like lesions in a photo‐distributed pattern is discussed.     

Recurrent and continuous erythema multiforme– a clinical and immunological study

A series of 26 patients with recurrent erythema multiforme was studied. A distinct clinical and immunological subgroup was found called ‘continuous erythema multiforme’ characterized by the presence of atypical lesions in addition to typical lesions, both of which occur continuously and a high level of circulating immune complexes with low levels of haemolytic complement. Herpes labialis preceded erythema multiforme in 17 of the 26 patients (65%) but in no cases could live virus be isolated from the lesions of erythema multiforme. Circulating immune complexes were found in 50 of 129 sera, being found in only 18 % sera between attacks and more commonly in the first 24 h of erythema multiforme lesions (58%). Immunological studies failed to provide conclusive evidence that erythema multiforme is solely immune complex mediated.     

Lupus erythematosus associated with erythema multiforme: report of two cases and review of the literature

Rowell's syndrome (RS) is a rare presentation of lupus erythematosus (LE) with erythema multiforme‐like lesions associated with antinuclear, anti‐La (SS‐B)/anti‐Ro (SS‐A) antibodies and rheumatoid factor (RF) positivity. This syndrome is suggested to be a different variant of cutaneous lupus erythematosus by some authors in literature. Here we present a 64‐year‐old woman with LE and a 51‐year‐old woman with LE and Sjögren syndrome (SS) who had erythema multiforme‐like eruptions and discuss the coexistence of lupus erythematosus and erythema multiforme.     

Persistent Erythema Multiforme Treated with Thalidomide

Erythema multiforme is a common self-limited disorder that predominantly affects younger individuals. It is characterized by typical iris or target lesions on the skin and mucous membranes. Three clinical subgroups of erythema multiforme have been identified: classical erythema multiforme, recurrent erythema multiforme, and persistent erythema multiforme. By definition, persistent erythema multiforme is characterized by the occurrence of continuous typical and atypical lesions without interruption. We report a 15-year-old boy who developed persistent erythema multiforme for 6 months and responded to treatment with thalidomide.     

Photocontact dermatitis to ketoprofen presenting with erythema multiforme

A 74-year-old Japanese man developed erythema multiforme on the inner aspect of his left elbow where ketoprofen-containing tape was applied and exposed to sunlight, and the eruption subsequently spread to the four limbs and trunk. The lesions were successfully treated with systemic corticosteroids without recurrence. Lymphocyte stimulation tests with ketoprofen-photomodified peripheral blood mononuclear cells revealed that the patient had circulating lymphocytes reactive with a photohaptenic moiety of ketoprofen. To our knowledge, this is the first case of erythema multiforme induced by photocontact dermatitis. The presence of circulating photoantigen-reactive T cells seemed to induce erythema multiforme as an unusual manifestation in this patient.     

ORF. CLINICAL AND EPIDEMIOLOGICAL STUDY

Twenty eight human cases of orf were studied from clinical and epidemiological points of view. Most of the patients were shepherds who were inoculated from infected animals during all seasons of the year. Typical lesions of different stages of orf were located on the hands, and were accompanied by local symptoms such as pain, pruritus, lymphangitis and adenitis, or less frequently by systemic symptoms such as fever or malaise. Two cases developed erythema multiforme, one developed erysipelas and another a papulovesicular eruption. Tzanck test may contribute to the diagnosis. The course of the disease can not be influenced by antibiotics, and only measures of local hygiene are recommended, except in complicated cases.     

Erythema multiforme

In summary, the diagnosis of erythema multiforme is appropriate for a self-limiting or episodic cutaneous or mucocutaneous illness with skin lesions morphologically and histologically compatible. With typical erythema multiforme minor, characterized by classic skin lesions with or without oral erosions, most patients' disease is associated with recurrent herpes simplex infections. This is particularly true with recurrent erythema multiforme. Symptomatic conservative care, antibiotic treatment for purulent secondarily infected oral lesions, and avoidance of systemic steroids are appropriate therapeutic guidelines. The more serious syndrome, erythema multiforme major, or Stevens-Johnson syndrome, is characterized by skin lesions that are somewhat atypical and different from those of erythema multiforme minor in association with erosions on multiple mucosal surfaces. Drugs and mycoplasmal infections are important precipitating factors for erythema multiforme major. Hospitalization and laboratory tests are often required because of the severity of the illness and the occasional damage to other organ systems. Conservative, symptomatic care, withdrawal of any drug that may have caused the illness, treatment of any mycoplasmal infection, and antibiotic therapy for purulent secondarily infected lesions are worthwhile therapeutic measures. Early treatment with systemic steroids may be helpful in preventing further damage, and the risks and potential benefits of such therapy must be evaluated on an individual basis.     

Identification of herpes simplex virus DNA in lesions of erythema multiforme by the polymerase chain reaction.

An association between erythema multiforme and herpes simplex virus infection has been supported by clinical studies and by the detection by immunofluorescence of herpes viral antigen in sera and skin biopsy specimens of patients with erythema multiforme. In rare cases, the virus has also been isolated in cultures of skin biopsy specimens of erythema multiforme. To investigate further the association between erythema multiforme and herpes simplex virus, we used the polymerase chain reaction for herpes simplex virus to examine skin lesions from patients with erythema multiforme. In this study herpes simplex virus DNA was detected in 11 of 31 biopsy specimens of erythema multiforme; six additional cases showed equivocal amplification results, which is suggestive of low amounts of viral DNA. Seven skin and mucosal biopsy specimens with the histologic changes of herpes virus infection served as positive controls: all were positive for herpes simplex virus DNA. Viral DNA was not detected in control biopsy specimens from skin excised for unrelated conditions. These studies support the association of herpes simplex virus in the pathogenesis of some cases of erythema multiforme. The polymerase chain reaction provides a quick and effective method of detecting herpes simplex virus in lesions of herpes-associated erythema multiforme. Furthermore, the polymerase chain reaction may delineate those cases of erythema multiforme that are etiologically related to herpes virus infection and therefore might be treated with acyclovir to prevent recurrence.     

Erythema multiforme: Differences between HSV‐1 and HSV‐2 and management of the disease—A case report and mini review

Erythema multiforme (EM) is an immune‐mediated reaction characterized by target lesions and with possible mucosal involvement. Its most frequent cause is HSV, with HSV‐1 more common than ?2. It is usually self‐limited but it can show recurrences. We report a peculiar case of recurrent herpes‐associated erythema multiforme (HAEM) in a 35‐year‐old man. The patient was affected by both herpes labialis and genitalis, but the typical target lesions were only associated with recurrent herpes labialis. Here, we hypothesize about the pathogenic differences between HSV‐1 and HSV‐2, and discuss the therapeutic management of HAEM.     

Human orf complicated by epidermolysis bullosa acquisita

Orf is a DNA parapoxvirus transmitted to humans by contact with infected goats and sheep. Many complications have been reported after orf infection, including erythema multiforme. A few cases of autoimmune bullous dermatosis complicating orf disease have been reported to date. They are usually characterized by tense blister eruptions with or without mucosal involvement; linear deposition of C3, IgG and/or IgA along the basement membrane; and negativity of indirect immunofluorescence analysis and enzyme‐linked immunosorbent assay (ELISA) (performed in four of 11 reported cases). These analyses have targeted antigens of bullous pemphigoid, mucous membrane pemphigoid or epidermolysis bullosa acquisita, except one case of mucosal pemphigoid with antilaminin‐332 antibodies. We describe the case of a patient who presented with an ulceration on his finger 10 days after direct contact with a lamb during Eid al‐Adha. Four weeks later he developed a severe tense blistering eruption associated with mucous membrane erosions. Indirect immunofluorescence analysis using the patient's serum revealed circulating antibasement membrane IgG that bound the dermal side of salt‐split skin. ELISA was positive for recombinant immunodominant NC1 domain of type VII collagen. We finally diagnosed epidermolysis bullosa acquisita complicating probable human orf infection.     

Isomorphic phenomenon in erythema multiforme

The cutaneous lesions of erythema multiforme have a propensity to localize at previously inflamed or traumatized skin sites. Evidence of the isomorphic phenomenon may be demonstrated in most cases of erythema multiforme by a careful history and physical examination. We present three cases of erythema multiforme which illustrate localization of skin lesions around scratches, recent surgical scars, lacerations, traumatized nail folds and folliculitis. The isomorphic phenomenon may serve as an important clue in understanding the clinical features and possibly, the pathogenesis of erythema multiforme.     

Oral acyclovir for the prevention of herpes-associated erythema multiforme

Herpes simplex virus is the single most common precipitator of erythema multiforme. Typically, erythema multiforme lesions appear 10 to 14 days after a recurrent herpes simplex virus infection and attacks can be disabling when they occur at frequent intervals. Prior to the introduction of acyclovir (Zovirax), there was no effective therapy to prevent herpes-associated erythema multiforme. Four patients were treated with a maintenance dose of acyclovir for periods ranging from 10 to 26 months; there were no significant side effects from the drug and only one recurrence of erythema multiforme. Oral acyclovir may become the treatment of choice for herpes-associated erythema multiforme.     

Erythema multiforme majus and Chlamydia pneumoniae infection

BACKGROUND: Erythema multiforme majus of infectious origin is an acute eruptive syndrome seen more commonly in young subjects and characterised by an appearance of round target lesions. In most cases, it is associated with infection involving Herpes simplex virus or Mycoplasma pneumoniae. We report an original case of erythema multiforme majus subsequent to infection with Chlamydia pneumoniae. CASE REPORT: An 18 year-old man was hospitalised for management of generalised skin rash comprising lesions in rings, associated with bullous and post-bullous lesions, chiefly in the oral (preventing eating) and genital areas in a setting of febrile cough. Various bacterial agents (Mycoplasma pneumoniae, Chlamydia pneumoniae) and viral agents were suspected, but serological testing for Chlamydia pneumoniae alone was positive with IgM of 128 IU and IgG of 64 IU. The outcome was favourable within several days following administration of symptomatic treatment (rehydration, mouthwashes, etc.) and aetiological treatment (acyclovir: 30 mg/kg/d, ofloxacine: 400 mg/d). At D15, serologic tests for Mycoplasma pneumoniae continued to be negative. Anti-Chlamydia pneumoniae IgM and IgG were 256 IU. At D30, IgM was 128 IU while IgG remained at 256 IU. DISCUSSION: The existence of a systematic skin rash comprising typical target lesions and mucosal lesions in the oral and genital areas suggested to us a diagnosis of erythema multiforme majus. Screening for the agents generally responsible was negative and drug-induced rash was ruled out. Serological tests for Chlamydia pneumoniae were positive at various times, resulting in diagnosis of erythema multiforme majus secondary to infection with Chlamydia pneumoniae. Following demonstration of the presence of Chlamydia pneumoniae using reliable methods and the elimination of other causes of erythema multiforme majus, dermatologists should opt for this aetiology in order to optimise treatment.     

Erythema multiforme,Stevens–Johnson syndrome and toxic epidermal necrolysis: Frozen‐section diagnosis

Stevens–Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) may be fatal. Although classified by body surface area skin detachment, initial stages of both may present with erythema multiforme (EM)‐like lesions. To diagnose and predict disease activity adequately as early as possible for patients revealing EM‐like lesions, we performed frozen‐section diagnosis. Thirty‐five patients clinically diagnosed as EM, SJS or TEN were biopsied to diagnose and predict disease progression within the initial‐visit day. Half of a histological section taken from a lesion was snap‐frozen and immediately cryostat‐sectioned, acetone‐fixed and stained with hematoxylin–eosin. Specimens were examined with light microscopy for presence of epidermal necrosis. A section from unaffected sites was also examined for 11 patients. Specimens were examined with light microscopy for presence of graft‐versus‐host reaction (GVHR)‐like findings: apoptotic keratinocytes and satellite cell necrosis. Epidermal necrosis was seen in nine patients. Initial diagnosis of the nine was one of overlap SJS‐TEN, four of SJS and four of EM, and final diagnosis of those was one of TEN, one of overlap SJS–TEN, four of SJS and three of EM. Dissociation between initial and final diagnosis was seen in three cases. GVHR‐like findings in the epidermis were observed in two patients finally diagnosed as overlap SJS–TEN and TEN. Frozen sections are useful not only to make a diagnosis of erythema multiforme but to assess a potential to exhibit more aggressive clinical behaviors (SJS or TEN).     

ERYTHEMA MULTIFORME INFANTUM ATROPHICANS

ABSTRACT: A new case of erythema multiforme is described, characterized by multiforme eruption of erythematous papules, plaques and occasional bullous lesions. The acute stage lasts several weeks, then atrophy is noted. The face appears senile. Histo-pathologically, the lesions show erythema multiforme in the acute phase; in the atrophic stage, a disappearance of the elastic fibers was observed.     

Treatment of extensive erythema multiforme with topical gentian violet

Topical and systemic therapies for erythema multiforme have been widely described in the literature. The pathogenesis of erythema multiforme involves increased expression of vascular endothelial growth factor resulting in the promotion of microvascular permeability and angiogenesis. Gentian violet has been shown to have antiangiogenic properties. Here, we present a case of erythema multiforme successfully treated with topical gentian violet. We report the case of a patient who presented with erythema multiforme. Prior pertinent history included diabetes mellitus type I, limiting the clinical use of systemic corticosteroids. Topical gentian violet was used to treat the cutaneous lesions. Our patient responded well to treatment with topical gentian violet with stabilization and resolution of the lesions without using systemic therapy that may pose serious side effects in the setting of other comorbidities such as diabetes mellitus type I. This case highlights the variable therapeutic options available for treatment of erythema multiforme, including topical gentian violet. While further studies are needed, this case demonstrates the antiangiogenic properties and clinical utility of topical gentian violet in the treatment of erythema multiforme.