Late onset pityriasis rubra pilaris type IV treated with low-dose acitretin

Pityriasis rubra pilaris is a chronic inflammatory dermatosis of unknown etiology and great clinical variability. It has been divided into six categories. Types III, IV, and V occur in childhood and are distinguished by their clinical presentation, age of onset, and course. We report a 19-year-old male patient with a 2-week history of pruritic, scaling dermatosis of the hands, feet, elbows, and knees. He had no family history of skin disease. On physical examination, we observed circumscribed, reddish-orange, scaling plaques affecting the elbows and knees and a waxy palmoplantar keratoderma. The skin biopsy showed acanthosis, alternating orthokeratosis, parakeratosis, and follicular plugging suggestive of pityriasis rubra pilaris. The patient started treatment with oral acitretin, 25 mg every other day. The treatment was tolerated well, and after 6 months the lesions had resolved completely. Pityriasis rubra pilaris is a chronic papulosquamous disorder of unknown pathogenesis, characterized by reddish-orange scaly plaques, palmoplantar keratoderma, and keratotic follicular papules. There is still no consensus regarding the treatment, but therapeutic options include systemic retinoids, particularly acitretin in the recommended dose of 0.5 to 0.75 mg/kg/day. In our case, the patient was treated with a low-dose regimen of acitretin, which was effective and well tolerated.info:eu-repo/semantics/publishedVersio

Cutaneous manifestations of antiphospholipid syndrome: a review of the clinical features, diagnosis and management

Antiphospholipid syndrome is a relatively recent systemic autoimmune disorder defined by thrombotic events and/or obstetric complications in the presence of persistent elevated antiphospholipid antibodies. It is characterized by a wide spectrum of clinical presentations and virtually any organ system or tissue may be affected by the consequences of vascular occlusion. Diagnosis is sometimes difficult and although classification criteria have been published and revised there remain ongoing issues regarding nomenclature, expanding clinical features, laboratory tests and management and much still has to be done. Cutaneous manifestations are common and frequently the first sign of the disease. Although extremely diverse it’s important to know which dermatological findings should prompt consideration of antiphospholipid syndrome and the appropriate management for those patients. Much has been debated about when to consider antiphospholipid syndrome and consensus still does not exist, however in spite of being a diagnostic challenge clinicians should know when to look for antiphospholipid antibodies since an early diagnosis is important to prevent further and serious complications. In this article we focus on the cutaneous features that should raise suspicion on the presence of antiphospholipid syndrome and on the complex management of such patients. Many other dermatological signs related to this syndrome have been described in the literature but only occasionally and without consistency or statistic impact and therefore will not be considered hereO síndrome antifosfolipidico é uma patologia auto-imune relativamente recente definida por eventos trombóticos e/ou complicações obstétricas na presença de anticorpos antifosfolípido elevados persistentes. Caracteriza-se por um vasto leque de apresentações clínicas e virtualmente qualquer sistema orgânico ou tecido pode ser afectado por oclusão vascular. O diagnóstico é por vezes difícil e, apesar de critérios de classificação terem sido publicados e revistos, muitas dúvidas persistem relativamente à nomenclatura, características clínicas, testes laboratoriais e abordagem destes doentes. As manifestações cutâneas são comuns e, frequentemente, o primeiro sinal da doença. Apesar da grande variabilidade, é importante reconhecer os achados dermatológicos que devem levantar a suspeita de um síndrome antifosfolipidico, assim como a abordagem adequada destes doentes. Muito tem sido debatido sobre quando considerar o diagnóstico desta entidade, não havendo ainda consenso neste assunto; no entanto, apesar de ser um desafio diagnóstico, é importante saber quando investigar a presença de anticorpos antifosfolípidos, uma vez que um diagnóstico precoce é crucial para prevenir complicações futuras graves. No presente artigo os autores descrevem as manifestações dermatológicas que devem fazer considerar o diagnóstico com enfoque na abordagem complexa destes doentes. Têm sido descritas na literatura muitas outras alterações cutâneas relacionadas com esta entidade, no entanto, apenas de forma ocasional e sem consistência ou impacto estatístico, pelo que não serão consideradas

Pyodermatitis vegetans of the vulva

Pyodermatitis vegetans (PV) is a rare inflammatory dermatosis of unknown etiology and difficult diagnosis characterized by vesiculopustular, exudative, and vegetating plaques usually localized in the axillary, genital, and oral region. It bears similarities to pemphigus vegetans but clinical history, histology, and immunofluorescence help confirm the diagnosis. Pyodermatitis vegetans is often associated with an underlying gastrointestinal disorder, especially ulcerative colitis. No standardized treatment plan is available for PV. Its evolution tends to follow the progression of the underlying disorder. We report a patient with vulvar PV with good response to systemic antibiotics and corticosteroids.info:eu-repo/semantics/publishedVersio

Erupção a fármaco com eosinofilia e sintomas sistémicos (síndrome dress)

Adverse cutaneous reactions to drugs are frequent, affecting from 2% to 3% of all hospitalized patients. But only about 2% of these cutaneous reactions are severe and seldom are fatal. The term drug hypersensitivity syndrome refers to a specific severe drug reaction, including skin rash, fever, lymph node enlargement, and single or multiple organ involvement. The cutaneous rash is usually morbilliform. The drugs associated with the syndrome are: anticonvulsants, ACE inhibitors, Beta-blockers, allopurinol and sulphonamides. The differencial diagnosis includes maculopapular rash, exfoliative dermatitis, acute generalized exanthematous pustulosis and Sézary syndrome. The interval between the starting of drug therapy and the onset of cutaneous reactions may be at least one month, and therefore the implication of the drug in the aetiology may be subdiagnosed. As reacções cutâneas a fármacos são frequentes, afectando 2 a 3% dos pacientes hospitalizados, mas só aproximadamente 2% destas são severas. O termo síndrome de hipersensibilidade a fármacos refere-se a uma reacção a fármacos caracterizada por erupção cutânea, febre, linfadenopatia e envolvimento de um ou mais órgãos sistémicos. As lesões cutâneas são normalmente morbiliformes. Os fármacos mais implicados neste tipo de reacções são os anticonvulsivantes, -bloqueadores, inibidores da enzima de conversão da angiotensina, alopurinol e as sulfamidas. O diagnóstico diferencial deverá ser feito com o exantema maculopapular, dermatite esfoliativa, pustulose exantemática aguda generalizada e com a síndrome de Sézary. As manifestações clínicas podem surgir até pelo menos um mês depois do início do fármaco, o que faz com que a implicação etiológica do mesmo possa ser subestimada. A morbilidade é alta e a mortalidade pode atingir os 10%, tornando importante o conhecimento desta patologia

Doença de Crohn vulvoperineal responsiva ao metronidazole

Crohn's disease is a multisystem chronic granulomatous inflammatory disease that primarily affects the gastrointestinal tract. In the majority of the cases, the cutaneous manifestations follow the intestinal disease, but occasionally dermatological lesions are the inaugural event and may constitute the only sign of the disease. Vulvoperineal involvement is rare, may precede bowel symptoms by months to years and may go unrecognized. Due to the paucity of reports of Crohn's disease at this location and in the absence of randomized trials, there are no standard treatments for the cutaneous disease. We describe the case of a 47 year-old woman with vulvoperineal Crohn's disease without digestive involvement, that was successfully managed with metronidazole

Vesico-bullous subacute cutaneous lupus erythematosus – An uncommon entity successfully treated with dapsone and hydroxychloroquine

Vesico-bullous subacute cutaneous lupus erythematosus is an uncommon and severe presentation. The authors report an exuberant case of vesico-bullous subacute cutaneous lupus erythematosus successfully treated with dapsone and hydroxychloroquin