Oral mucosa lesions as atypical manifestation of adult-onset Still´s disease

Abstract: Adult-onset Still's disease is a systemic inflammatory disorder of unknown etiology, characterized by skin rash, spiking fever, arthralgias or arthritis, and leukocytosis. The typical skin rash is evanescent, salmon-pink, nonpruritic and maculopapular, predominantly on the extremities. It is considered one of the major Yamaguchi's criteria in adult-onset Still's disease. However, atypical skin lesions are also described. Here, a 61-year-old woman with sore throat, spiking fever, polyarthritis and evanescent salmon-pink nonpruritic maculopapular skin rash on the extremities was diagnosed with adult-onset Still's disease. In addition, atypical brown macules on oral mucosa, localized on the inner lips and tongue were also observed. Biopsy revealed a neutrophilic infiltrate. Despite treatment and improvement of the adult-onset Still's disease, the atypical oral mucosal lesions persisted.

Abordagem cutânea na necrólise epidérmica tóxica / Cutaneous approach in toxic epidermal necrolysis

Introdução: Necrólise epidérmica tóxica é uma erupção mucocutânea aguda grave, geralmente induzida por medicamentos, associada a alta taxa de morbidade e mortalidade. Os cuidados com as lesões mucosas e cutâneas e a abordagem multidisciplinar são muito importantes para o prognóstico e sequelas futuras. Objetivos: Discutir os principais aspectos dessa síndrome por meio da revisão de literatura, ilustrada por um caso clínico. Métodos: Revisão de literatura utilizando bases de dados on-line PubMed e Scielo. Incluímos artigos em língua inglesa, portuguesa, francesa e espanhola, e ilustração com caso clínico pediátrico. Termos procurados foram "toxic epidermal necrolysis", "Stevens-Johnson overlap", ''necrólise epidérmica tóxica'', ''síndrome Stevens-Johnson''. Resultados: Apresentamos dados para guiar o manejo de pacientes com necrólise epidérmica tóxica para cirurgiões plásticos, pediatras, intensivistas, dermatologistas e emergencistas. O caso tratado teve evolução favorável, sem sequelas cutâneas. Conclusão: O alto nível de suspeição é imprescindível para um diagnóstico e estratificação de risco adequados e instituição precoce de medidas de suporte, e o tratamento deve ser realizado por uma equipe multidisciplinar treinada para reduzir sequelas e mortalidade.

Introduction: Toxic epidermal necrolysis is a severe acute mucocutaneous condition usually induced by drugs associated with a high rate of morbidity and mortality. The care of the mucous lesions and skin and a multidisciplinary approach are very important for the prognosis and future sequelae. Objectives: To discuss the main aspects of this syndrome through a literature review illustrated by a clinical case. Methods: Review of the literature using the PubMed and SciELO online databases was performed. Articles in English, Portuguese, French, and Spanish were included and illustrated with a pediatric clinical case. The keywords used were as follows: "toxic epidermal necrolysis," "Stevens-Johnson overlap," "necrólise epidérmica tóxica," and "síndrome Stevens-Johnson." Results: We presented data to guide the management of patients with toxic epidermal necrolysis for plastic surgeons, pediatricians, intensivists, dermatologists, and emergency physicians. The case treated had a favorable disease course without sequelae. Conclusion: A high level of suspicion is necessary for an adequate diagnosis and risk stratification, and early support measures and treatment should be performed by a multidisciplinary team trained to minimize damage and mortality.

Confluent and reticulated papillomatosis subsiding spontaneously after delivery: report of one case / Papilomatosis confluente y reticulada que remite espontáneamente después del parto: informe de un caso

Gougerot and Carteaud confluent and reticulated papillomatosis (CARP) is an uncommon dermatosis characterized by hyperpigmented scaly macules or papillomatous papules coalescing into confluent patches or plaques centrally with a reticular pattern peripherally. We report a 28-year-old woman presenting at 16 weeks of gestation with an itchy rash that was biopsied and turned out to be consistent with CARP. Options for treatment were discussed but the woman refused to take any systemic therapy and used only moisturizers throughout her pregnancy. The rash subsided spontaneously after delivery.

Gianotti-Crosti syndrome: a case report of a teenager

Abstract Gianotti-Crosti syndrome is a rare disease characterized by acral papular eruption with symmetrical distribution. It is a benign and self-limited disease; the symptoms disappear after two to eight weeks, without recurrences or scars. Skin lesions are usually asymptomatic. Prodrome might occur, suggesting upper respiratory infection, or constitutional symptoms. Diagnosis is eminently clinical, and this disease is associated with viral infections. Due to its rarity and low occurrence in adolescents and adults, we report a case of Gianotti-Crosti syndrome of a teenager.

Acute generalized exanthematous pustulosis associated with spider bite

Abstract: Acute generalized exanthematous pustulosis (AGEP) is an acute febrile rash, usually induced by drugs, which recently has been linked to spider bite. We report a case of a male patient, 48 years old, with an erythematous rash accompanied by fever and small non-follicular pustules. He reported previous pain in the buttock with the onset of a necrotic plaque. The lesion was compatible with spider bite of the genus Loxosceles. According to the EuroSCAR group instrument, the patient scored +10 indicating definite diagnosis of AGEP. As the patient had a compatible lesion and had no other triggers of AGEP, in an Loxosceles endemic area, the AGEP would be associated with spider bite, as described in other publications.

Atypical cutaneous features in adult onset Still's disease.

Adult onset Still's disease is a rare but potentially serious disease. We present five cases of adult-onset Still's disease seen by us over a period of one year. The patients were all females and 28-39 years of age. Symptoms had been present for 2-6 weeks in three patients. The other two had been on a few years' follow-up for rheumatoid arthritis before the onset of rashes and fever. The patients had persistent erythematous maculopapular eruptions on face, body and extremities, with moderate to severe pruritus and/or a burning sensation that decreased their quality of life. The typical evanescent rash was not observed. High ferritin values were detected in all the patients and total serum IgE was increased in two. All the patients were started on oral prednisolone (0.5-1.0 mg/kg/day), and methotrexate (10-15 mg/week) had to be added in three patients. One patient was started on tocilizumab due to recalcitrant disease and one was lost to follow-up. Further investigation and classification of the various atypical cutaneous findings in adult-onset Still's disease is necessary.

Enfermedad de manos, pies y boca en paciente adulto / Disease hand, foot and mouth in an adult patient

La enfermedad de manos, pies y boca es causada por el virus coxsackie A es extremadamente contagiosa y de duración limitada. Es característica de niños y muy eventualmente en adultos. Se asocia a fiebre, malestar general, linfoadenopatías tras las cuales aparece una erupción vesicular localizada en la boca que se rompen originando úlceras superficiales y se acompañan de la aparición de pápulas eritematosas en las palmas de las manos, en las plantas de los pies, que pasan a vesículas y luego se ulceran. No requiere tratamiento específico, es autolimitada, con un tiempo de duración de 1 a 2 semanas. Se presenta un caso de paciente femenina de 52 años de edad quien consulta por presentar lesiones ulcerativas en la cavidad bucal acompañada con sintomatología general. Antecedentes familiares y personales no contributorios. La paciente refiere inicio de la enfermedad actual hace aproximadamente 6 días cuando aparecieron lesiones vesiculo - ulcerativas en la mucosa bucal, concomitante con fiebre y malestar general. Refiere haber tenido contacto con una nieta que presentó el mismo cuadro clínico. No ha recibido tratamientos anteriores. Al examen clínico extrabucal se observa en la piel de las manos y en los pies lesiones papulomatosas y algunas ulcerativas, que causan molestias a la paciente. Al examen intrabucal se observa lesiones de naturaleza ulcerativa ubicadas en mucosa de los carrillos y pilar anterior amigdalino. Se le indico tratamiento sintomático. Se destaca la importancia de este caso en su presentación clínica ya que es una enfermedad frecuente en la infancia y siendo inusual en pacientes de edad adulta

Hand, foot and mouth disease is caused by the virus coxsackie A, is extremely contagious and of limited duration. Is typical of children and very eventually in adults. It associates to fever, general discomfort, linfoadenopatía after which a vesicular eruption appears located in the mouth, they break originating superficial ulcers and they accompany with the appearance of wheal and flare in the palms of the hands, in the plants of the feet, which go on to bladders and then ulcerate. It does not need specific treatment, is autolimited, with a time of duration from 1 to 2 weeks. It is presented a case of 52-year-old female patient of age, who consults for presenting ulcerative injuries in the oral cavity accompanied with general symptomatology. Family and personal precedents are not contributers. The patient recounts the beginning of the current disease approximately 6 days ago when appeared ulcerative bladders injuries in mucous mouth, concomitant with fever and general discomfort. She recounts to have had contact with a granddaughter who presented the same clinical picture. She has not received previous treatments. In the clinical extrabuccal examination is observed in the hand's skin and in the feet papulomatosas and some ulceratives injuries, which cause inconveniences to the patient. In the intrabuccal examination is observed injuries of ulcerative nature located in mucous of the pulleys and previous prop amigdalina. It was indicate symptomatic treatment. Is outlined the importance of this case in its clinical presentation since it is a frequent disease in the infancy and being unusual in patients of adult age

A Case of Mexiletine-induced Hypersensitivity Syndrome Presenting as Eosinophilic Pneumonia

An 82-yr-old man was presented with fever and cough accompanied by generalized erythematous rash. He had taken mexiletine for 5 months, as he had been diagnosed with dilated cardiomyopathy and ventricular arrhythmia. Laboratory studies showed peripheral blood eosinophilia and elevated liver transaminase levels. Chest radiographs showed multiple nodular consolidations in both lungs. Biopsies of the lung and skin lesions revealed eosinophilic infiltration. After a thorough review of his medication history, mexiletine was suspected as the etiologic agent. After discontinuing the mexiletine and starting oral prednisolone, the patient improved, and the skin and lung lesions disappeared. Subsequently, mexiletine was confirmed as the causative agent based on a positive patch test. Drug-induced hypersensitivity syndrome is a severe adverse reaction to drugs and results from treatment with anticonvulsants, allopurinol, sulfonamides, and many other drugs. Several cases of mexiletine-induced hypersensitivity syndrome have been reported in older Japanese males with manifestation of fever, rash, peripheral blood eosinophilia, liver dysfunction without other organ involvement. Here, we report a case of mexiletine-induced hypersensitivity syndrome which presented as eosinophilic pneumonia in a Korean male.

Síndrome de Ramsay Hunt: relato de caso / Ramsay Hunt Syndrome: case report

A Síndrome de Ramsay Hunt é definida por paralisia facial periférica acompanhada de um rash eritematosoe vesicular no pavilhão auricular ou na boca, causado por herpes zoster. Comparado com a paralisia de Bell,os pacientes com Síndrome de Ramsay Hunt geralmente apresentam um quadro de paralisia mais grave e dificilmentese recuperam totalmente. Alguns estudos sugerem que o tratamento com aciclovir e predinisona possa melhorar o prognóstico, entretanto estudos randomizados ainda são necessários. Os autores descrevem um caso desta síndrome acompanhado de ceratopatia de exposição.

Ramsay Hunt Syndrome consists in facial peripheric paralysis associated with auricular or mouth rash andvesicles, caused by herpes zoster. Compared to Bell’s paralysis, the patients with Ramsay Hunt Syndrome usually present worse paralisy and hardly have a total recovery. Some studies suggest that acyclovir and prednisone treatment may improve the prognosis, but random control studies are still necessary. The authors describeone case of this syndrome with exposure keratopathy.

Clinical Features of Polyarteritis Nodosa in Korea

Polyarteritis nodosa (PAN) is a systemic vasculitis characterized by multi-organ involvement with protean manifestations. We evaluated the clinical features of PAN in Korea. Twenty-seven patients were diagnosed as PAN at Seoul National University Hospital between January 1990 and July 2003. The male-to-female ratio was 1.7:1 and mean age at onset (+/-SD) was 47.4+/-20 yr. Their presenting features at diagnosis were similar to those reported previously, i.e., myalgia, muscle weakness or leg tenderness (70%), fever (52%), weight loss >4 kg (44%), skin rash (44%), peripheral edema (33%), abdominal pain (33%), and arthralgia/arthritis (30%). However, the prevalence of testicular pain or tenderness was higher (24%) than reported previously and only three (11.5%) had HBsAg positivity without liver enzyme elevation. Nine patients (33%) had a five-factor score (FFS) of 2. Fourteen patients (52%) responded to treatment, 2 patients relapsed and 4 died within 1 yr of diagnosis. During a median follow-up of 55.5 months, three of the four PAN-related deaths had an initial FFS of 2. The clinical features of PAN were not significantly different from those reported previously. However, testicular pain or tenderness was more frequent and patients with a high FFS tended to have a poorer prognosis.

Acute generalized exanthematous pustulosis (AGEP): case report

A pustulose exantemática aguda generalizada (AGEP) é uma dermatose induzida por droga, caracterizada por episódio agudo de aparecimento de pústulas estéreis sobre base eritemato-edematosa, acompanhado de febre. Este quadro regride espontâneamente após a suspensão da droga ou em resultado do tratamento com corticóide sistêmico. As principais drogas envolvidas são agentes antifúngicos, antiinflamatórios não hormonais, analgésicos, antiarrítmicos e anticonvulsivantes. Histologicamente caracteriza-se por vasculite associada a pústulas subcórneas não foliculares. Relatamos caso de paciente branca, feminina, que se apresentou com lesões pustulosas generalizadas após o uso de cefalosporina. O diagnóstico foi confirmado pelos achados clínicos e histológicos; pela resolução do quadro após a suspensão da droga e pela introdução de corticóide sistêmico, e pela recorrência após a introdução de droga similar. A importância do reconhecimento deste tipo de dermatose induzida por droga reside na necessidade importante de seu diagnóstico diferencial, clínico e histológico, com a psoríase pustulosa generalizada e a pustulose subcórnea, particularmente no que tange às opções terapêuticas que se apresentam para o tratamento das mesmas.

Síndrome da sulfona e reaçäo reversa / Sulfone syndrome and reverse reaction

Um homem de 68 anos de idade, cor preta, interna-se por hanseníase multibacilar, nao tratada, apresentando infiltraçao cutânea difusa e nódulos raros de Eritema nodoso hansênico. Na época, (1982) foi tratado com sulfona e talidomida. Um mês e meio após o início do tratamento surge erupçao cutânea eritematosa difusa, acompanhada de febre alta, adenomegalia generalizada e hepatoesplenomegalia. O hemograma demonstrou leucocitose intensa com linfocitose e atipias de linfócitos. Evoluiu com icterícia, desidrataçao, confusao mental, falecendo no 4( dia após instalaçao da erupçao referida. Na necrópsia o substrato do exantema generalizado é um infiltrado linfocitário pleiomórfico em pele, mucosas e vísceras. Este quadro foi interpretado como a "Síndrome da Sulfona", manifestaçao de hipersensibilidade à esta droga e que em muitos aspectos mimetiza ou induz o aparecimento de uma monucleose infecciosa. Há algumas publicaçoes sobre esta síndrome, algumas relatando casos fatais, más nao há descriçao de achados necroscópicos. Ao lado do infiltrado linfocitário atípico observou-se também reaçao granulomatosa tuberculóide generalizada englobando infiltrado de padrao virchoviano com baciloscopia rica. Os autores acreditam que este quadro representaria uma reaçao reversa induzida pela "Síndrome da Sulfona".