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5.
Medicine (Baltimore) ; 98(46): e17895, 2019 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-31725635

RESUMEN

RATIONALE: Erythema multiforme (EM) is an immune-mediated disease with mucocutaneous localization and plurietiologic determinism. The term "multiforme" refers to the variety of aspects that the lesions can take from patient to patient and during evolution in a single patient. PATIENT CONCERNS: We have selected 2 cases of small children diagnosed with different etiology of EM to illustrate the importance of a correct and fast diagnosis. Case 1 involves a 2-year-old girl from a rural area who presented with fever and pruritic erythematous papular eruption. The onset of the symptoms was 3 days before presentation with fever and ulcerative lesions on the oral and labial mucosa, followed by the appearance of erythematous macular lesions, with progressive confluence to intense pruritic patches. The 2nd involves a 2-year-old boy with fever, loss of appetite, productive cough, and petechiae. He had corticosensible immune thrombocytopenia from the age of 6 months, with many recurrences. The patient received treatment with ampicillin/sulbactam and symptomatics for an erythemato-pultaceous angina. During the 2nd day of treatment the patient developed an erythematous macular eruption on the face, scalp, trunk, and limbs, with bullae formation. DIAGNOSES: The 1st patient was diagnosed based on biologic findings: positive inflammatory syndrome, elevated level of anti-Mycoplasma pneumoniae immunoglobulin M antibodies and immunoglobulin E. Histopathologic examination described papillary dermal edema, inflammatory infiltrate, and lymphocyte exocytosis. In the 2nd case, the hemoleucogram identified 12,000/mm platelets and the medulogram aspect was normal. Serology for Epstein-Barr virus was negative. The diagnosis was EM secondary to M pneumoniae infection in case 1 and secondary to administration of ampicillin/sulbactam in case 2. INTERVENTIONS: In both cases, etiopathogenic treatment consisting of steroidal antiinflammatory drugs, antihistamines was administered. Because of specific etiology, the 1st case received antibiotics. OUTCOMES: The evolution was favorable in 10 to 14 days; the patients were discharged after etiopathogenic treatment consisting of steroidal antiinflammatory drugs, antihistamines, and/or antibiotics. LESSONS: Performing a detailed clinical examination, medical history of drug use, infection or general diseases can establish a good diagnosis of EM. Histopathologic examination can help. The treatment is etiologic, pathogenic, and symptomatic. EM usually has a self-limited evolution.


Asunto(s)
Eritema Multiforme/diagnóstico , Eritema Multiforme/fisiopatología , Antibacterianos/uso terapéutico , Antiinflamatorios/uso terapéutico , Preescolar , Eritema Multiforme/tratamiento farmacológico , Eritema Multiforme/microbiología , Femenino , Antagonistas de los Receptores Histamínicos/uso terapéutico , Humanos , Masculino , Rumanía
6.
Lupus ; 28(14): 1716-1721, 2019 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-31674268

RESUMEN

Rowell syndrome (RS) is a rare disease characterized by the association of systemic lupus erythematosus (SLE) or cutaneous lupus with lesions similar to erythema multiforme and the presence of autoantibodies including ANA, SSA, SSB, or rheumatoid factor. Due to the low incidence of this disease, the epidemiology of RS is not clear. So far there are 95 cases reported in the literature; of these, only seven cases are pediatric patients. Macrophage activation syndrome (MAS) is an increasingly recognized complication of SLE, although its true prevalence in childhood is still unknown. We describe a unique pediatric patient with RS who developed MAS.


Asunto(s)
Eritema Multiforme/diagnóstico , Lupus Eritematoso Sistémico/diagnóstico , Síndrome de Activación Macrofágica/etiología , Piel/patología , Niño , Diagnóstico Diferencial , Eritema Multiforme/patología , Humanos , Lupus Eritematoso Sistémico/patología , Masculino
9.
Ugeskr Laeger ; 181(43)2019 Oct 21.
Artículo en Danés | MEDLINE | ID: mdl-31617477

RESUMEN

Stevens-Johnson syndrome is an autoimmune condition characterised by erythematous target lesions on the skin with involvement of the oral and genital mucosa and conjunctivae. Recent case reports describe incomplete presentations with absence of the characteristic skin changes. This is caused by extrapulmonary manifestations of Mycoplasma pneumoniae lung infection and is now recognised as M. pneumoniae-associated mucositis (MPAM). This case report describes the clinical presentation of MPAM in a 17-year-old girl and highlights the importance of the recognition and the appropriate clinical management.


Asunto(s)
Eritema Multiforme , Mucositis , Neumonía por Mycoplasma , Adolescente , Eritema Multiforme/etiología , Femenino , Humanos , Mucositis/etiología , Mycoplasma pneumoniae , Neumonía por Mycoplasma/complicaciones
10.
Acta Dermatovenerol Croat ; 27(3): 200-201, 2019 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-31542069

RESUMEN

Dear Editor, Systemic lupus erythematosus (SLE) is a heterogeneous autoimmune disease characterized by diverse patterns of auto-antibody production with multi-organ affectation. Cutaneous involvement, either alone or in association with other systemic illnesses, is one of its most common manifestations (1). Dermatologic disorders like malar and discoid rashes are quite suggestive of SLE. However, the occurrence of non-specific skin lesions like erythema multiforme (EM) in patients with SLE (Rowell syndrome) can rarely occur (1). In such patients, a diagnosis of SLE may be missed or delayed in the absence of other overt clinical features of lupus. Herein we report a case of recurring EM-like eruptions as the cardinal cutaneous manifestation of previously undiagnosed, active SLE in a young Nigerian woman. A 26-year-old Nigerian woman presented with a three-day history of non-pruritic, generalized, and target-like, erythematous annular patches and plaques which mostly affected the trunk. A few lesions had presented with crusting and erosions at the time of examination (Figure 1). Associated symptoms included oral painful ulcers, low grade fever, and malaise. The patient had no other systemic symptoms and her prior drug history was not remarkable. Her erythrocyte sedimentation rate (ESR) was 66 mm/hour using the Westergren method. Screening for HIV and hepatitis B and C was negative. Herpes simplex, cytomegalovirus, and Epstein Barr viruses could not be screened for. Other baseline investigations (complete blood count, electrolytes, urea and creatinine as well as urinalysis) were within normal limits. The patient was managed as a case of EM of an unidentified inciting agent and her symptoms resolved with supportive care and antibiotics. However, she developed a recurrence about 5 weeks later, with more extensive and coalescent skin lesions (Figure 2). Additionally, there was a new onset of alopecia and pain in the small joints of the hands as well as both knees and ankles. At this time, the patient's ESR had gone up to 112 mm/h and she had developed significant proteinuria, with a protein creatinine ratio of 1.3 g/g (reference <0.5 g/g). Her antinuclear antibody (ANA) titer was high (1:320) with a speckled pattern. Anti-Smith antibody was also positive. A renal biopsy was declined. A tentative diagnosis of Rowell syndrome was made. The patient was started on high-dose steroids and hydroxychloroquine 200 mg twice daily. Subsequent care included the use of mycophenolate mofetil 1 g twice daily for 6 months. This was then changed to azathioprine at 50 mg twice daily. Follow-up after 6 months showed sustained clearance of skin lesions, resolution of fever and joint pains, as well as improvement in the renal profile, with a urine protein-creatinine ratio of 0.77 g/g. The presence of systemic lupus erythematosus, EM-like lesions, and a speckled pattern of antinuclear antibody in our patient fulfils the revised diagnostic criteria for RS put forward by Zeitouni et al. at the turn of the twenty-first century (2). Considering the rarity of EM-like lesions in SLE and the possibility of constitutional symptoms in EM, a diagnosis of RS may be readily overlooked in patients like the one described, whose major cutaneous manifestation of severe active SLE was EM-like lesions. In contrast to classic EM, where skin lesions are concentrated in the extremities, a predominant truncal distribution of EM-like lesions as found in our patient may favor a clinical consideration of RS (3). However, some authors have challenged the existence of Rowell syndrome as a distinct clinical laboratory entity. Arguments put forward in this regard include the fact that none of the immunological markers that have been described in RS are specific to any disorder. Additionally, the annular polycyclic dermatosis seen in sub-acute cutaneous lupus erythematosus (SCLE) can be difficult to clinically and histologically differentiate from EM (4,5). Patients with SLE also have a higher likelihood of developing adverse drug reactions (6). The inherent complexity of SLE may make for delayed and oftentimes difficult diagnosis, especially in a country where immunologic tests are expensive and rheumatologists are scarce. When patients do occasionally present with recurrences of skin lesions in the spectrum of EM, Steven-Johnson syndrome, and toxic epidermal necrolysis in the absence of a definite inciting agent, undiagnosed lupus may indeed be present in some of these individuals and should be considered in the differential diagnosis. In conclusion, while it is very rare, SLE may present first with recurrent episodes of EM-like rash. Despite the various possibilities which underlie their association, prompt identification and treatment of SLE in patients presenting with EM is important to prevent death or irreversible organ damage.


Asunto(s)
Eritema Multiforme/diagnóstico , Eritema Multiforme/etiología , Lupus Eritematoso Sistémico/complicaciones , Lupus Eritematoso Sistémico/diagnóstico , Adulto , Femenino , Humanos , Nigeria
13.
BMJ Case Rep ; 12(8)2019 Aug 20.
Artículo en Inglés | MEDLINE | ID: mdl-31434669

RESUMEN

Good's syndrome (GS) is a rare, adult-onset combined B cell and T cell immunodeficiency with an associated thymoma. These patients have an increased risk of bacterial, fungal, viral and opportunistic infections. This report describes a 75-year-old female patient who presented with a full body rash and an anterior mediastinal mass. She underwent a biopsy of her rash and mass, which revealed erythema multiforme and WHO Type A thymoma, respectively. During her hospitalisation, she was also found to have oropharyngeal candidiasis, methicillin-susceptible Staphylococcus aureus bacteraemia and herpes simplex virus type 2 (HSV-2) skin lesions. Based on the number of infections and severity of her rash, an immunocompromised state was suspected. Immunological testing revealed a B cell and T cell deficiency as well as low serum immunoglobulins. This combination of hypogammaglobulinaemia and thymoma led to a diagnosis of GS. While there have been many case reports of GS, this is the first report of the immunodeficiency presenting with erythema multiforme.


Asunto(s)
Antibacterianos/uso terapéutico , Rehabilitación Cardiaca , Eritema Multiforme/diagnóstico , Síndromes de Inmunodeficiencia/diagnóstico , Infecciones Estafilocócicas/diagnóstico , Timoma/diagnóstico , Combinación Trimetoprim y Sulfametoxazol/uso terapéutico , Anciano , Eritema Multiforme/complicaciones , Eritema Multiforme/tratamiento farmacológico , Eritema Multiforme/inmunología , Resultado Fatal , Femenino , Fluidoterapia , Humanos , Huésped Inmunocomprometido , Síndromes de Inmunodeficiencia/complicaciones , Síndromes de Inmunodeficiencia/tratamiento farmacológico , Síndromes de Inmunodeficiencia/inmunología , Neumonía , Choque Séptico , Infecciones Estafilocócicas/tratamiento farmacológico , Infecciones Estafilocócicas/inmunología , Timoma/inmunología
14.
Dermatol Ther ; 32(5): e13066, 2019 09.
Artículo en Inglés | MEDLINE | ID: mdl-31414706

RESUMEN

Herpes simplex virus (HSV)-associated erythema multiforme (HAEM) is an acute and self-limiting mucocutaneous hypersensitivity reaction triggered by herpes virus infections. We reported a patient with HAEM after hematopoietic stem cell transplantation (HSCT). A 55-year-old man received HSCT 7 months ago. He suffered from chronic graft versus host disease 4 months after HSCT and was treated with prednisone and tacrolimus. One week ago, he developed generalized macules with leukopenia. Dermatological examination revealed multiple iris-like erythemas on his trunk and extremities. The skin lesions and leukopenia resolved upon anti-HSV treatment.


Asunto(s)
Eritema Multiforme/virología , Famciclovir/administración & dosificación , Enfermedad Injerto contra Huésped/terapia , Trasplante de Células Madre Hematopoyéticas/efectos adversos , Herpes Simple/patología , Simplexvirus/aislamiento & purificación , Biopsia con Aguja , Eritema Multiforme/tratamiento farmacológico , Eritema Multiforme/patología , Estudios de Seguimiento , Enfermedad Injerto contra Huésped/diagnóstico , Herpes Simple/etiología , Humanos , Inmunohistoquímica , Masculino , Persona de Mediana Edad , Síndromes Mielodisplásicos/patología , Síndromes Mielodisplásicos/terapia , Medición de Riesgo , Simplexvirus/efectos de los fármacos , Factores de Tiempo , Resultado del Tratamiento
15.
BMJ Case Rep ; 12(8)2019 Aug 12.
Artículo en Inglés | MEDLINE | ID: mdl-31409617

RESUMEN

A 69-year-old man with esophageal EBV-positive diffuse large B cell lymphoma status post allogeneic bone marrow transplant (BMT) five months prior presented to his oncologist with three days of maculopapular rash that was initially diagnosed as grade 1 graft-versus-host disease and started on oral prednisone. However, due to worsening of the rash, the patient presented to dermatology clinic, where skin biopsy revealed a diagnosis of erythema multiforme (EM). The patient improved with the use of topical steroids. This case highlights the atypical morphology of post-BMT EM and the potential causes for this atypical appearance.


Asunto(s)
Trasplante de Médula Ósea/efectos adversos , Eritema Multiforme/etiología , Exantema/etiología , Linfoma de Células B/terapia , Anciano , Humanos , Masculino
16.
J Am Acad Dermatol ; 81(3): 813-822, 2019 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-31331726

RESUMEN

BACKGROUND: Erythema multiforme (EM) is an acute inflammatory mucocutaneous condition. EM is rarely described in children and infants. OBJECTIVE: To investigate the triggers, clinical manifestations, and treatment of pediatric EM. METHODS: Systematic literature review of pediatric EM. RESULTS: After full-text article review, we included 113 articles, representing 580 patients. The mean age was 5.6 years, ranging 0.1-17 years. Infectious agents were the main triggers: herpes simplex virus (HSV) in 104 patients (17.9%) and Mycoplasma pneumoniae in 91 patients (15.7%). In total, 140 cases (24.1%) were drug-related and 89 cases (15.3%) had other triggers, such as vaccines (19 patients, 3.2%). In total, 229 patients had EM major (39.5%). Treatment was supportive care only (180 patients, 31.1%), systemic corticosteroids (115 patients, 19.8%), antivirals (85 patients, 14.6%), and antibiotics (66 patients, 11.3%), mostly macrolides (45 patients, 7.7%). Long-term sequelae were rare (1.3%). Pediatric EM was reported in 19 infants (3.2%). The main trigger was vaccination (9 patients). Infantile EM was EM major in 2 cases and EM minor in 17. Infants were less prone to develop EM major than older children (P < .01). Pediatric EM was recurrent in 83 cases (14.3%), which was triggered by HSV in 36 patients (61%). Recurrence affected older children. LIMITATIONS: Potential confusion between Steven Johnson syndrome and EM major in addition to publication bias. CONCLUSION: Pediatric EM is a rare disease, mainly triggered by infections. This condition can affect all mucosal surfaces, most commonly the oral mucosae. The diagnosis is clinical, and management relies on supportive care. Vaccines are a particular trigger in infants. Recurrent cases are most commonly linked to HSV. Dermatologists and pediatricians should be aware of this potentially recurrent and severe condition.


Asunto(s)
Eritema Multiforme/etiología , Cuidados Paliativos/métodos , Enfermedades Raras/etiología , Vacunación/efectos adversos , Adolescente , Factores de Edad , Antibacterianos/uso terapéutico , Antivirales/uso terapéutico , Niño , Preescolar , Diagnóstico Diferencial , Erupciones por Medicamentos/diagnóstico , Erupciones por Medicamentos/etiología , Erupciones por Medicamentos/terapia , Eritema Multiforme/diagnóstico , Eritema Multiforme/terapia , Glucocorticoides/uso terapéutico , Herpes Simple/complicaciones , Herpes Simple/tratamiento farmacológico , Humanos , Lactante , Mucosa Bucal/microbiología , Mucosa Bucal/patología , Neumonía por Mycoplasma/complicaciones , Neumonía por Mycoplasma/tratamiento farmacológico , Enfermedades Raras/diagnóstico , Enfermedades Raras/terapia , Recurrencia , Índice de Severidad de la Enfermedad
18.
Acta Dermatovenerol Croat ; 27(2): 124-126, 2019 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-31351509

RESUMEN

Dear Editor, Rowell's syndrome is a rare disease, characterized by the appearance of erythema multiforme (EM)-like lesions in patients with lupus erythematosus. It was initially reported by Rowell (1) in 1963 and its existence as a separate clinical entity is currently under debate (2,3). A few cases may have been induced by drugs such as systemic antimycotics, antibiotics, anticonvulsants, and more recently proton pump inhibitors (PPIs). CASE REPORT We present the case of a 67-year-old woman with subacute cutaneous lupus erythematosus (SCLE) and EM-like lesions who fulfilled all the criteria for Rowell's syndrome. The patient had lupus arthritis for two years and was treated with oral methylprednisolone 8 mg/day and hydroxychloroquine 200 mg/day. She started receiving 20 mg of omeprazole daily for gastroprotection. The patient also had arterial hypertension with no current treatment, osteoporosis, and an L1 vertebral fracture. The dermatological examination revealed multiple erythematous infiltrated plaques involving mainly the sun-exposed areas (neck, chest, upper back, and shoulders). Cutaneous lesions had an annular or target pattern and a tendency to form hemorrhagic crusts and scales at the margins (Figure 1, A). The mucous membranes were unaffected. Histological examination (hematoxylin and eosin ×200) found epidermal atrophy, vacuolar degeneration of the basal layer, and sparse perivascular lymphocytic infiltrate in the dermis - features corresponding to lupus erythematosus (Figure 2, A). Single eosinophilic necrotic keratinocytes characteristic for erythema multiforme were observed in the epidermis (Figure 2, B). Direct immunofluorescence (IF) from lesional skin showed granular deposits of C3 on the dermo-epidermal junction. Lupus band test from sun-protected, nonlesional skin was negative. On indirect IF a speckled pattern antinuclear antibodies (ANA) with >1:1280 titers were detected. Anti-Ro (>200 U/mL) and anti-La (>200 U/mL) antibodies were also positive. The blood cell count and differential analysis were within reference ranges. The 24-hour urine protein test showed a non-significant proteinuria - 0.36 g/24h. Photo-testing was impossible considering the extent of the skin lesions. The therapeutic approach consisted of increasing the hydroxychloroquine dose to 400 mg/day, substituting PPI with famotidine 20 mg/day p.o. and ceftriaxone 2 g/day for the superinfection with Ps. aeruginosa, which led to a clinical improvement (Figure 1, B). The methylprednisolone dose remained unchanged due to already existing severe adverse effects. DISCUSSION The diagnosis was based on Zeitouni et al.'s classification (4). The three main criteria are as follows: lupus erythematosus, EM-like lesions, and speckled pattern of ANA. Our patient met all three major and one minor criteria, namely the presence of anti-Ro and anti-La antibodies. As for the other minor criteria, RF was negative and no chilblains were found. Although there was a continuous time lapse (more than 1 year) between the initiation of omeprazole intake and the diagnosis of Rowell's syndrome, we suggest that the connection is probable. For instance, the latency differs depending on the incriminated medication in drug induced SCLE. Longer periods are reported for diuretics and calcium blockers, while the time interval is shorter for chemotherapeutic drugs and antimycotics (5). Our suspicions were further confirmed by the fact that the lesions improved promptly within a month after discontinuation of omeprazole and doubling the dose of hydroxychloroquine. PPIs are reported to be a major cause of drug-induced SCLE (6,7). According to Laurinaviciene et al., the most common drugs involved are PPIs, thiazide diuretics, antifungals, chemotherapeutics, statins, and antiepileptics (6). However, very few cases of Rowell's syndrome are found to be drug-related. The culprit drugs include: oral terbinafine (8,9), norfloxacin (10), sodium valproate (11) and esomeprazole (12) (Table 1). CONCLUSION Despite the common clinical and immunological features shared between SCLE, drug-induced SCLE and EM, Rowell's syndrome seems to be a separate entity rather than a coincidental association. Finally, according to our knowledge this case would be the second of Rowell's syndrome due to PPIs.


Asunto(s)
Eritema Multiforme/inducido químicamente , Lupus Eritematoso Cutáneo/tratamiento farmacológico , Omeprazol/efectos adversos , Inhibidores de la Bomba de Protones/efectos adversos , Anciano , Eritema Multiforme/tratamiento farmacológico , Femenino , Humanos , Síndrome
19.
Am Fam Physician ; 100(2): 82-88, 2019 07 15.
Artículo en Inglés | MEDLINE | ID: mdl-31305041

RESUMEN

Erythema multiforme is an immune-mediated reaction that involves the skin and sometimes the mucosa. Classically described as target-like, the erythema multiforme lesions can be isolated, recurrent, or persistent. Most commonly, the lesions of erythema multiforme present symmetrically on the extremities (especially on extensor surfaces) and spread centripetally. Infections, especially herpes simplex virus and Mycoplasma pneumoniae, and medications constitute most of the causes of erythema multiforme; immunizations and autoimmune diseases have also been linked to erythema multiforme. Erythema multiforme can be differentiated from urticaria by the duration of individual lesions. Erythema multiforme lesions are typically fixed for a minimum of seven days, whereas individual urticarial lesions often resolve within one day. Erythema multiforme can be confused with the more serious condition, Stevens-Johnson syndrome; however, Stevens-Johnson syndrome usually contains widespread erythematous or purpuric macules with blisters. The management of erythema multiforme involves symptomatic treatment with topical steroids or antihistamines and treating the underlying etiology, if known. Recurrent erythema multiforme associated with the herpes simplex virus should be treated with prophylactic antiviral therapy. Severe mucosal erythema multiforme can require hospitalization for intravenous fluids and repletion of electrolytes.


Asunto(s)
Eritema Multiforme/diagnóstico , Eritema Multiforme/terapia , Eritema Multiforme/etiología , Humanos
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