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Severe cutaneous drug reactions include a wide spectrum of clinical manifestations ranging from mild morbilliform cutaneous rash, to severe forms of hypersensitivity. Special attention is given in this report to the acute generalized exanthematous pustulosis (AGEP), induced in 90% of cases by the use of systemic drugs, especially aminopenicillins and macrolides. The incidence of the disease is low, 1-5 cases per million patients / year. The main differential diagnosis is Von Zumbusch's Pustular Psoriasis. The prognosis is generally good and the disease self limited, after withdrawal of the triggering drug. In this report the authors describe a case of AGEP, triggered by ceftriaxone in a patient with psoriasis vulgaris.

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Darier's disease is characterized by dense keratotic lesions in the seborrheic areas of the body such as scalp, forehead, nasolabial folds, trunk and inguinal region. It is a rare genodermatosis, an autosomal dominant inherited disease that may be associated with neuropsichiatric disorders. It is caused by ATPA2 gene mutation, presenting cutaneous and dermatologic expressions. Psychiatric symptoms are depression, suicidal attempts, and bipolar affective disorder. We report a case of Darier's disease in a 48-year-old female patient presenting severe cutaneous and psychiatric manifestations.

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AbstractSevere cutaneous drug reactions include a wide spectrum of clinical manifestations ranging from mild morbilliform cutaneous rash, to severe forms of hypersensitivity. Special attention is given in this report to the acute generalized exanthematous pustulosis (AGEP), induced in 90% of cases by the use of systemic drugs, especially aminopenicillins and macrolides. The incidence of the disease is low, 1-5 cases per million patients / year. The main differential diagnosis is Von Zumbusch's Pustular Psoriasis. The prognosis is generally good and the disease self limited, after withdrawal of the triggering drug. In this report the authors describe a case of AGEP, triggered by ceftriaxone in a patient with psoriasis vulgaris.

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Traumatic neuromas are tumors resulting from hyperplasia of axons and nerve sheath cells after section or injury to the nervous tissue. We present a case of this tumor, confirmed by anatomopathological examination, in a male patient with history of circumcision. Knowledge of this entity is very important in achieving the differential diagnosis with other lesions that affect the genital area such as condyloma acuminata, bowenoid papulosis, lichen nitidus, sebaceous gland hyperplasia, achrochordon and pearly penile papules.

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Abstract Traumatic neuromas are tumors resulting from hyperplasia of axons and nerve sheath cells after section or injury to the nervous tissue1. We present a case of this tumor, confirmed by anatomopathological examination, in a male patient with history of circumcision. Knowledge of this entity is very important in achieving the differential diagnosis with other lesions that affect the genital area such as condyloma acuminata, bowenoid papulosis, lichen nitidus, sebaceous gland hyperplasia, achrochordon and pearly penile papules.

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Darier’s disease is characterized by dense keratotic lesions in the seborrheic areas of the body such as scalp, forehead, nasolabial folds, trunk and inguinal region. It is a rare genodermatosis, an autosomal dominant inherited disease that may be associated with neuropsichiatric disorders. It is caused by ATPA2 gene mutation, presenting cutaneous and dermatologic expressions. Psychiatric symptoms are depression, suicidal attempts, and bipolar affective disorder. We report a case of Darier’s disease in a 48-year-old female patient presenting severe cutaneous and psychiatric manifestations.

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Trichilemmoma is a benign neoplasm from the outer sheath of the pilosebaceous follicle. Desmoplastic trichilemmoma, a rare variant, is histologically characterized by a central area of desmoplasia that can clinically mimic an invasive carcinoma, requiring histopathological examination to define the diagnosis.

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Hansen's disease is a chronic infecto-contagious disease caused by Mycobacterium leprae. The bacillus prefers low-temperature areas and the nose is usually the initial site of lesions. Transmission of the bacilli occurs by nasal and oropharyngeal secretions, and through solutions of continuity of the skin and/or mucosae. Nasal manifestations are found in the later stages of the disease.

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Focal acral hyperkeratosis is a rare genodermatosis with an autosomal dominant pattern of inheritance. It is characterized by usually asymptomatic keratotic papules along the borders of the hands and/or feet. The main differential diagnosis is acrokeratoelastoidosis of Costa, which differs from the former only by not presenting elastorrhexis in histopathological examination, thus requiring this exam for a correct diagnosis.

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Focal acral hyperkeratosis is a rare genodermatosis with an autosomal dominant pattern of inheritance. It is characterized by usually asymptomatic keratotic papules along the borders of the hands and/or feet. The main differential diagnosis is acrokeratoelastoidosis of Costa, which differs from the former only by not presenting elastorrhexis in histopathological examination, thus requiring this exam for a correct diagnosis.

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Hansen's disease is a chronic infecto-contagious disease caused by Mycobacterium leprae. The bacillus prefers low-temperature areas and the nose is usually the initial site of lesions. Transmission of the bacilli occurs by nasal and oropharyngeal secretions, and through solutions of continuity of the skin and/or mucosae. Nasal manifestations are found in the later stages of the disease.

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Trichilemmoma is a benign neoplasm from the outer sheath of the pilosebaceous follicle. Desmoplastic trichilemmoma, a rare variant, is histologically characterized by a central area of desmoplasia that can clinically mimic an invasive carcinoma, requiring histopathological examination to define the diagnosis.

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Rendu-Osler-Weber Syndrome also known as Hereditary Hemorrhagic Telangiectasia is a rare systemic fibrovascular dysplasia, with dominant autosomal inheritance. It is characterized by recurrent epistaxis, mucocutaneous telangiectasia, visceral arteriovenous malformation and positive family history. There may be hematologic, neurologic, dermatologic and gastrointestinal complications. Therapy is supportive and aimed at preventing complications. In this article we report a case of Rendu-Osler-Weber in a 64 year-old man, with history of mucocutaneous telangiectasia since the third decade of life, recurrent epistaxis, positive family history and vascular ectasia in the gastrointestinal tract.

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Rendu-Osler-Weber Syndrome also known as Hereditary Hemorrhagic Telangiectasia is a rare systemic fibrovascular dysplasia, with dominant autosomal inheritance. It is characterized by recurrent epistaxis, mucocutaneous telangiectasia, visceral arteriovenous malformation and positive family history. There may be hematologic, neurologic, dermatologic and gastrointestinal complications. Therapy is supportive and aimed at preventing complications. In this article we report a case of Rendu-Osler-Weber in a 64 year-old man, with history of mucocutaneous telangiectasia since the third decade of life, recurrent epistaxis, positive family history and vascular ectasia in the gastrointestinal tract.

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Cutis verticis gyrata is characterized by excessive formation of scalp skin. It may be primary (essential and nonessential) or secondary. In the primary essential form it presents only folding skin formation on the scalp, mimicking cerebral gyri, without associated comorbidities. We report a rare case of a 28 year-old male patient with primary essential cutis verticis gyrata.

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Cutis verticis gyrata is characterized by excessive formation of scalp skin. It may be primary (essential and nonessential) or secondary. In the primary essential form it presents only folding skin formation on the scalp, mimicking cerebral gyri, without associated comorbidities. We report a rare case of a 28 year-old male patient with primary essential cutis verticis gyrata.

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Linear immunoglobulin A dermatosis is a rare autoimmune bullous disease, but the most common autoimmune bullous dermatosis in children. We report a typical exuberant case of linear IgA dermatosis in a ten-month old child, who showed good response to treatment with corticosteroids and dapsone.

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Linear immunoglobulin A dermatosis is a rare autoimmune bullous disease, but the most common autoimmune bullous dermatosis in children. We report a typical exuberant case of linear IgA dermatosis in a ten-month old child, who showed good response to treatment with corticosteroids and dapsone.

A dermatose por imunoglobulina A (IgA) linear é doença bolhosa autoimune rara, porém a mais comum das dermatoses bolhosas autoimunes da infância. Relatamos caso típico e exuberante de dermatose por IgA linear em uma criança de 10 meses, que apresentou boa evolução com o tratamento com corticóide e dapsona.

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Introdução: Tumores neurogênicos constituem uma porcentagemmuito baixa entre das neoplasias de cabeça e pescoço. Dentreos schwanomas extracranianos, aproximadamente 25% a45% ocorrem nessa topografia, sendo a localização maiscomum a região lateral do pescoço. A incidência é baixa naregião oromaxilofacial, sendo 1% de origem intra-oral. Entreesses últimos, a língua e o soalho da boca são os sítios maiscomumente afetados e, menos frequentemente, o palatoduro. Quando acomete esse sítio, um diagnóstico diferencialimportante são os tumores de glândulas salivares menores, muitomais frequentes nessa localização. Objetivos: Descrever umcaso de schwanoma intra-oral, mais especificamente localizadono palato duro, e discutir o diagnóstico e tratamento desta lesãorara. Relato do caso: Relata-se um caso de um schawanoma depalato duro tratado em nosso serviço cuja suspeita diagnósticainicial era de tumor de glândula salivar. Os aspectos histológicose achados imuno-histoquímicos são apresentados e discutidos àluz da literatura consultada. Considerações finais: Descreve-seum caso raro de schwanoma intra-oral localizado no palato duro.Dada a maior prevalência de tumores de glândulas salivaresnesta topografia, o exame imuno-histoquímico é essencial paraum diagnóstico preciso permitindo um tratamento adequadodessa doença.

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O fibroqueratoma digital adquirido é tumor fibroepitelial benigno, raro, que, tipicamente, se apresenta como nódulo solitário assintomático nos quirodáctilos e pododáctilos. Relata-se o caso clínico de uma paciente do sexo feminino que apresentava duas lesões de fibroqueratoma digital no quarto e no segundo quirodáctilos esquerdos.

Acquired digital fibrokeratoma is a rare benign fibroepithelial condition, which typically occurs as a solitary asymptomatic nodule in fingers and toes. The authors report the clinical case of a female patient affected by two digital fibrokeratomas in the 4th and 2nd left fingers, respectively.