Erythema Multiforme-like Lesions Research Articles

Overlap syndrome with rowell's syndrome, antiphospholipid syndrome, primary sterility, and sensorineural hearing loss: A case report, brief review, and analysis of cases of rowell's syndrome reported from India and Abroad

Rowell's syndrome (RS) is recognized since 1963 as a presentation of lupus erythematosus (LE) with erythema multiforme-like lesions and characteristic immunological changes. Antiphospholipid syndrome (APS) encompasses antibodies to phospholipids/phospholipid-binding cofactor proteins and/or circulating lupus anticoagulant with clinical manifestations of thrombosis such as recurrent spontaneous abortions, etc. A 32-year-old female with primary infertility since marriage (7 years) and sequential appearance, for the past 6 months, of various lupus-specific cutaneous lesions, “targetoid” lesions on palms, musculoskeletal, ocular and auditory (sensorineural hearing loss [SNHL]) complaints with positive serological profile for SMD-1, SS-A/Ro, SS-B/La, U1-snRNP, Ku, antiphospholipid and anticardiolipin antibodies, and histopathologically confirmed LE is presented as a case of concurrent overlap syndrome, RS and APS, primary infertility, and SNHL. A brief review of RS including analysis of data of cases reported from India hereto vis-a-vis that of cases worldwide up to 2012 has also been done.

Drug-induced Rowell syndrome, a rare and difficult to manage disease: A case report.

Rowell syndrome is defined as the association between lupus erythematosus, erythema multiforme-like lesions and characteristic immunological changes including positive tests for rheumatoid factor, speckled antinuclear antibody, positive anti-Ro or anti-La antibodies. The present report presents the case of a 45-year-old female patient who was previously diagnosed in January 2010 with terbinafine-induced subacute cutaneous lupus erythematosus and was admitted for a skin eruption consisting of erythematous-papular erythema multiforme-like lesions, primarily on the trunk and limbs. The associated symptoms consisted of fatigability, myalgia and gonalgia. In October 2015, the illness reoccurred ~1 week after the initiation of Helicobacter pylori eradication treatment. Anti-Ro antibodies, rheumatoid factor and antinuclear antibody tests were positive. Given the patient's medical history, clinical manifestations, and laboratory, histopathological and immunofluorescence microscopy findings, a diagnosis of Rowell syndrome was made. Systemic corticosteroids (methylprednisolone; 0.5 mg/kg/day) and immunomodulatory therapy (azathioprine; 50 mg/day) were administered with the associated medication (omeprazole, 20 mg/day; KCl, 1 g/day) and topical dermocorticoids (fluticasone propionate 0.05% cream; 1 application/day), with a favorable outcome. The major diagnostic criteria for Rowell syndrome are the presence of lupus erythematosus (acute, subacute or systemic), erythema multiforme-like lesions and positive testing for antinuclear antibodies. The minor diagnostic criteria for Rowell syndrome are chilblains, the presence of anti-Ro antibodies and positive testing for rheumatoid factor. A diagnosis of Rowell syndrome is made if the patient exhibits all major criteria and at least one minor criterion. The present case met all diagnostic criteria, excluding the presence of chilblains. Notably, in this case there was a co-occurrence of subacute lupus erythematosus and Rowell syndrome lesions, which was drug-induced.

P73: CONTACT ALLERGY TO HAIR DYES (P‐PHENYLENEDIAMINE): A PRELIMINARY REPORT

Hair dye is a popular cosmetic product nowadays. Many strong and extreme sensitising chemicals, such as para-phenylenediamine (PPD), toluene-2,5-diamine (TDA) and other aromatic amines or cross-reacting substances, are ingredients in hair dye products. To study the clinical patterns and PPD contact sensitivity in patients with hair-dye dermatitis. Ninety five (M:F 56:39) consecutive patients aged between 18 and 70 years suspected to have contact allergy from hair dye were studied by patch testing standard patch test series including p-phenylenediamine (PPD, 1.0% pet). Seventy-six (M:F 39:36) patients showed positive patch tests from PPD. Twenty-one of these patients also showed positive patch test reaction from fragrance mix, thiuram mix, paraben mix, or colophony. Fifty-six (74%) patients affected were aged older than 40 years. The duration of dermatitis varied from <1 month to >1 year with exacerbation following hair coloring. Fifty-three (70%) patients had dermatitis of scalp and/or scalp margins and 22 patients (30%) had face and neck dermatitis. Periorbital dermatitis, chronic actinic dermatitis, and erythema multiforme-like lesions were seen in 6, 3, and 2 patients, respectively. Hair dyes and PPD constitute a significant cause of contact dermatitis. There is an urgent need for creating consumer awareness regarding hair-dyes contact sensitivity and the significance of performing sensitivity testing prior to actual use.

Rowell Syndrome: A case report and review of literature

Rowell syndrome is a rare disease consisting of lupus erythematosus associated with erythema multiforme-like lesions. The present case study discusses the occurrence of this syndrome in a 38-year-old female who presented with erythematous scaly lesions on face, neck and back, along with the targetoid lesions on upper arms and back of the neck, with oral erosions. Laboratory investigations revealed speckled pattern of anti-nuclear antibody (ANA) with a titer of 1:640, and anti-dsDNA and anti-La (SS-B) positivity. Histology of the targetoid lesion was consistent with erythema multiforme. Significant improvement within four weeks was noted with the use of oral steroids and anti-malarials.

ROWELL SYNDROME – A CONTROVERSIAL CLINICAL ENTITY

Rowell syndrome is considered to be an unusual association between systemic lupus erythematous (SLE), erythema multiforme-like lesions and an immunological pattern consisting of speckled ANA, positive rheumatoid factor and positive anti-La (SSB) antibodies. We present the case of an 81 year old woman with late-onset SLE who developed an erythema multiforme-like rash, with a speckled antinuclear antibodies (ANA) immunofluorescence pattern and a positive rheumatoid factor. The key in establishing the diagnosis was the biopsy of the lesion, which set the diagnosis of Rowell syndrome.

Contact dermatitis: relevant differential diagnoses, simulators, and variants.

Similar to all eczematous disorders, irritant and allergic contact dermatitis are primarily defined by their morphology. In addition, the diagnosis of this specific reaction pattern on the skin (and mucous membranes) also requires prior exposure (direct or indirect) to an allergen or irritant. While it is quite easy to give a textbook description of the typical features of eczema, its clinical manifestations in daily practice are diverse and frequently uncharacteristic. Contact reactions may present as lichenoid, lymphomatoid, granulomatous, pigmented, purpuric, and erythema multiforme-like lesions, thus lacking the typical eczematous appearance and broadening the spectrum of differential diagnoses to be considered. Moreover, a considerable number of agents, acting by mechanisms other than contact, may trigger adverse reactions mimicking the eczematic morphology and distribution pattern of contact dermatitis. Increasingly used in oncology, this is especially true for drugs such as kinase inhibitors. Knowledge of these associations is crucial for the adequate diagnostic and therapeutic care of patients with supposed or actual contact dermatitis.

Hair dye dermatitis and p-phenylenediamine contact sensitivity: A preliminary report.

Background:The contact allergic reactions from p-phenylenediamine (PPD) in hair dyes vary from mild contact dermatitis to severe life- threatening events (angioedema, bronchospasm, asthma, renal impairment).Objectives:To study the clinical patterns and PPD contact sensitivity in patients with hair-dye dermatitis.Materials and Methods:Eighty (M:F 47:33) consecutive patients aged between 18 and 74 years suspected to have contact allergy from hair dye were studied by patch testing with Indian Standard Series including p-phenylenediamine (PPD, 1.0% pet).Results:54 Fifty-four (M:F 21:33) patients showed positive patch tests from PPD. Eight of these patients also showed positive patch test reaction from fragrance mix, thiuram mix, paraben mix, or colophony. Fifty-seven (71%) patients affected were aged older than 40 years. The duration of dermatitis varied from < 1 month to > 1 year with exacerbation following hair coloring. Forty-nine patients had dermatitis of scalp and/or scalp margins and 23 patients had face and neck dermatitis. Periorbital dermatitis, chronic actinic dermatitis, and erythema multiforme-like lesions were seen in 4, 2, and 1 patients, respectively.Conclusions:Hair dyes and PPD constitute a significant cause of contact dermatitis. There is an urgent need for creating consumer awareness regarding hair-dyes contact sensitivity and the significance of performing sensitivity testing prior to actual use.

Rowell's Syndrome to ds-DNA Negative Lupus Nephritis: A Yet Unreported Progression.

Rowell's syndrome is now identified as a subtype of subacute lupus erythematosus (LE) with erythema multiforme-like skin lesions, positive serum rheumatoid factor, anti-Ro La positivity and speckled pattern of antinuclear antibodies. Here we describe a case of Rowell's syndrome in an 18-year-old female who was found to be ds-DNA negative, who later progressed to develop stage V lupus nephritis (LN) over a course of 4 months. Although extremely rare, most cases of LN are drug induced. Of only seven cases of non-drug induced LN with negative dsDNA, none had cutaneous involvement. Ours was a unique case of progression of Rowells syndrome to ds-DNA negative LN.

Pityriasis rosea with erythema multiforme - like lesions: An observational analysis

Pityriasis rosea (PR) is an acute or subacute inflammatory skin disease characterized by erythematous papulosquamous eruptions localized on the trunk and arms. The eruptions are self-limiting and usually disappear gradually in 2-10 weeks, without any treatment. Typical PR is much easier to diagnose than the rare atypical forms. There is a passing mention of PR with erythema multiforme-like lesions in the literature, but no extensive case series have been published till date. We present a series of five patients for whom we believe atypical PR is the likely diagnosis.

English

Leprosy is an ancient disease that has survived into the modern ages despite an intense effort to eliminate it worldwide.[1] Here we report a case of recurrent, multiple ulcerative lesions with pitting edema of both lower extremities, in a 62 years old male. He was a diagnosed case of Hansen’s disease with irregular treatment since 2 years. All biochemical parameters were within normal limits. Rheumatoid factor, ANA, ELISA for HIV and VDRL were negative. Modified ZN staining (using 5% sulphuric acid) shows acid fast bacilli (grading +++), arranged in globi, 65% were fragmented & 35% were uniformly stained. Organisms isolated in bacterial culture were Proteus mirabilis & Staphylococcus aureus & Candida spp isolated in fungal culture. Patient was admitted and was started on MDT-MB along with prednisolone. KEY-WORDS: Erythema nodosum leprosum, Mycobacterium leprae, Modified ZN stain INTRODUCTION: Leprosy is an ancient infectious disease caused by Mycobacterium leprae that affects the skin and peripheral nerves. A wide clinical spectrum of disease exists, from the tuberculoid pole [associated with a vigorous Th1 response, relatively few bacilli, and limited well-defined lesions] to the lepromatous pole [associated with aggressive Th2 response, many bacilli, and diffuse symmetric lesions].[2] Though it is a chronic disease, it has tendency for debilitating acute exacerbations termed reactions; Type 1 Reaction [Reversal Reaction] and Type 2 Reaction [Erythema Nodosum Leprosum (ENL)].[1] Erythema nodosum leprosum, is an acute inflammatory reaction seen in patients with lepromatous leprosy or occasionally in borderline lepromatous leprosy.[3] Though it is usually seen during the course of treatment it may occur in previously untreated patients as well.[4] The lesions are erythematous painful tender papules and nodules. In mild reaction nodules are small in number and spontaneously resolve leaving behind hyperpigmented macules.[5] In severe reactions, nodules tend to increase in size and ulcerate. Vesiculobullous, pustular, ulcerated, and hemorrhagic and erythema multiforme-like lesions have been reported in ENL.[6] In this article, we have discussed a case which presented to us with recurrent, multiple ulcerative lesions with pitting edema of both lower extremities. The case was admitted in our hospital, Pradumna Bal Memorial Hospital, KIMS, Bhubaneswar, where he was investigated, diagnosed and started on treatment. CASE REPORT: A 62 year old male was presented with multiple painful ulcerated skin lesions over both the lower extremities and gluteal areas of 3 months duration. He had history of recurrent attacks of papulo-pustular lesions, and ulcerations since two years. The skin lesion

Immunopathogenic characterization of cutaneous inflammation in Behçet’s disease

Behçet's disease (BD) is a recurrent multisystemic inflammatory disease characterized by recurrent oral aphthous and genital ulcers, ocular lesions and cutaneous lesions. Although many studies of cytokine levels in sera of BD patients have been conducted, there are only limited number of studies about the cytokine expression and cellular infiltration in the BD-related skin lesions. To investigate the immunophenotypes and cytokine profiles of BD-related skin lesions. Twenty patients who fulfilled the diagnostic criteria for BD with BD-related skin lesions were enrolled in this study. We assessed the histopathological features of BD-related skin lesions by immunohistochemical studies with anti-human CD4, CD8, CD68, FoxP3, CD-11b, IFN-γ and IL-4 antibodies. Immunophenotyping of inflammatory infiltrating cells showed that CD68+ macrophages were the most common type of infiltrated cells in erythema nodosum-like lesions, erythema multiforme-like lesions and Sweet's syndrome-like lesions, whereas neutrophils were the main population of inflammatory infiltrating cells in papulopustular lesions. In all of the four types of BD-related skin lesions, the percentage of CD8+ T cells was higher than that of CD4+ T cells (P < 0.05), and IL-4 expression was stronger than IFN-γ expression (P < 0.05). In this study, we assessed the infiltrating inflammatory cells and cytokine expression of acute cutaneous lesions in BD through immunohistochemical staining of BD-related skin lesions. Further studies about the disease activity and the molecular biology underlying the cutaneous inflammation are needed to understand the detailed pathogenesis of BD.

Psoriatic erythroderma coexisting with erythema multiforme-like lesions induced by retinoids or retinoids combined with an antibiotic: case report

Psoriasis is currently considered a multifactorial disease, which can coexist with many somatic and psychological disorders. We present the case of a 50-year-old woman referred to our department due to erythroderma with concomitant peculiar violaceous, polycyclic lesions most likely induced by medications. Past medical history revealed numerous systemic disorders, including metabolic syndrome, hypertension, cardiac insufficiency, obesity, and depression. Additional examinations and consultations demonstrated dyslipidemia, xanthelasma, incomplete block of the right branch of His bundle, thyreocardiac syndrome, benign adrenal tumor, and delusions. Recently, psoriasis has been intensively studied. We present the case in which erythroderma was most likely triggered by acitretin combined with ceftriaxone. Treatment of many diseases and psychiatric disturbances coexisting with psoriasis is extremely difficult and requires cooperation of various specialists.

Erythema Multiforme-like Irritant Contact Dermatitis after Application of an Antiscabies Treatment

We describe the case of an irritant contact dermatitis due to an antiscabies treatment in a man who presented to our clinic with an important cutaneous reaction with many hemorrhagic, "target" erythema multiforme-like lesions, as the result of an acute toxic insult of the skin by permethrin 5%. This is a possible, but very uncommon symptom of non-eczematous contact dermatitis and an unusual drug causing the acute hypersensitivity reaction typical of erythema multiforme.

Rowell’s syndrome: presenting features of systemic lupus erythematosus

A subset of patients with lupus erythematosus develops erythema multiforme-like skin lesions with speckled pattern of antinuclear antibodies, positive rheumatoid factor, anti-Ro (SS-A), and anti-La antibodies (SS-B), which known as Rowell's syndrome. We report an adolescent boy presented with erythema multiforme-like skin lesions and pericardial effusion; he fulfilled criteria of both Rowell's syndrome and systemic lupus erythematosus (SLE). Such initial presentation of SLE is rarely reported in literature.

Erythema multiforme-like lesions in the course of infectious mononucleosis

The rash in infectious mononucleosis is usually diffusely macular. A 15-year-old boy presented to us with high grade fever, sore throat, malaise, body aches, and polyarthralgia. He developed annular, erythematous, and non-scaly eruptions on chest and right arm. Blanching erythema was noted on his trunk. He had bilateral tender cervical lymph nodes, severe pharyngeal congestion, petechiae on soft palate, uvular edema, infraorbital edema, and marginal tender hepatomegaly. Investigations revealed lymphocytosis and activated atypical lymphocytes in the peripheral smear, and positive monospot test. The boy subsequently recovered in one week with total disappearance of his rash. Epstein-Barr virus-related infectious mononucleosis was considered the most likely diagnosis for our patient. To our knowledge, this atypical case is the third reported case of annular lesions in infectious mononucleosis. Dermatologists and other clinicians should be alerted to this special presentation of primary EBV infection.

Annular eruption preceding relapsing polychondritis: case report and review of the literature

Relapsing polychondritis (RP) is a rare multisystem disorder affecting cartilaginous tissues, primarily of the ears, nose, larynx, and trachea. RP shows dermatologic manifestations in 35-50% of cases, and may show skin findings as the first sign of disease in 12% of affected patients. There have been reports of urticarial-like lesions in RP, typically associated with vasculitis, as well as a few reports of erythema multiforme-like lesions. There has also been one previous report of erythema annulare centrifugum associated with RP, presenting with a histology suggestive of a drug eruption. Our patient presented with urticarial and erythema annulare centrifugum-like plaques preceding the diagnosis of RP by over 1 year, with an initial histology suggestive of a drug eruption.

Leflunomide-induced subacute cutaneous lupus erythematosus with erythema multiforme-like lesions

Leflunomide is an immunosuppressive agent that acts by inhibiting pyrimidine synthesis in lymphocytes and other rapidly proliferating cells, as well as by suppressing tumor necrosis factor-alpha-induced cellular responses. A number of leflunomide-related adverse events have been reported. Among cutaneous side effects, a few cases of subacute cutaneous lupus erythematosus have been described. We report a previously undocumented reaction to leflunomide, manifesting as subacute cutaneous lupus erythematosus and erythema multiforme-like lesions, in a young woman treated with this drug for ankylosing spondylitis. Withdrawal of leflunomide combined with a short cycle of systemic corticosteroid led to the resolution of the patient's rash, indicating this drug as being responsible for the development of the disease. We conclude that leflunomide might have triggered the occurrence of both subacute cutaneous lupus erythematosus and erythema multiforme in a patient with pre-existing autoimmune diathesis. The suppressive effect of this drug on tumor necrosis factor-alpha-related mechanisms might have played a role in the induction of such a unique reaction to leflunomide.

Rowell syndrome - case report and review of the literature

Rowell syndrome is a rare distinctive entity first described in 1963 by Rowell and coworkers. It consists in erythema multiforme-like lesions associated with lupus erythematosus. Zeitouni et al. recently redefined the major and minor diagnostic criteria. Major criteria are lupus erythematosus, erythema multiforme-like lesions and speckled pattern of antinuclear antibody. Minor criteria are chilblains, positive anti-La (SS-B) or anti-Ro (SS-A) antibody and reactive rheumatoid factor. We report a case of a 63-year-old woman, with no previous history of lupus erythematosus, who developed erythema multiforme-like lesions and laboratory findings consistent with Rowell syndrome.

Index of Suspicion

Index of Suspicion

Systemic lupus erythematosus with an erythema multiforme-like lesions

Patients with lupus erythematous may develop an acute eruption clinically similar to toxic epidermal necrolysis or erythema multiforme. The presence of erythema multiforme-like lesions and characteristic pattern of immunological abnormalities including antinuclear antibody (speckled pattern), anti-Ro antibody or anti-La antibody and positive rheumatoid factor in lupus patients has been termed as Rowell's syndrome. Although diagnostic criteria of this syndrome have been reviewed recently, definite mechanisms of pathogenesis is still unknown. Here we reported a 29-year-old female patient who had systemic lupus erythematosus developed erythema multiforme-like lesions.<br>