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.

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.

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.

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.

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.

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.

FUNDAMENTO: A queilite actínica crônica é a ceratose actínica localizada no vermelhão labial. O tratamento é de crucial importância, devido ao potencial de transformação maligna. OBJETIVO: Avaliar os resultados estéticos e funcionais das vermelhectomias clássica e em W-plastia na queilite actínica. Na técnica clássica, a cicatriz é linear; na W-plastia, em linha quebrada. MÉTODOS: Foram tratados 32 pacientes com diagnóstico clínico e histopatológico de queilite actínica. Quinze deles foram submetidos à técnica em W-plastia e 17, à técnica clássica. Avaliaram-se parâmetros como retração cicatricial e alterações funcionais. RESULTADOS: Houve associação estatisticamente significativa entre a técnica utilizada e a presença de retração cicatricial, sendo a associação positiva com a clássica (p=0,01 com correção de Yates). O risco relativo (odds ratio - OR) calculado foi de 11,25, ou seja, houve maior chance de retração nos pacientes submetidos à técnica clássica. Nenhuma das técnicas apresentou alterações funcionais. Avaliaram-se complicações pós-operatórias como presença de crostas, lábios secos, parestesia e deiscência de sutura. Não houve associação estatisticamente significante entre as complicações e a técnica utilizada (p=0,69). CONCLUSÃO: Concluiu-se que a vermelhectomia em W-plastia oferece melhores resultados estéticos e índices de complicações semelhantes.

BACKGROUND: Chronic actinic cheilitis is actinic keratosis located on the vermilion border. Treatment is essential because of the potential for malignant transformation. OBJECTIVE: To evaluate the aesthetic and functional results of vermilionectomy using the classic and Wplasty techniques in actinic cheilitis. In the classic technique, the scar is linear and in the W-plasty one, it is a broken line. METHODS: 32 patients with clinical and histopathological diagnosis of actinic cheilitis were treated. Out of the 32 patients, 15 underwent the W-plasty technique and 17 underwent the classic one. We evaluated parameters such as scar retraction and functional changes. RESULTS: A statistically significant association between the technique used and scar retraction was found, which was positive when using the classic technique (p = 0.01 with Yates' correction). The odds ratio was calculated at 11.25, i.e., there was a greater chance of retraction in patients undergoing the classic technique. Both techniques revealed no functional changes. We evaluated postoperative complications such as the presence of crusts, dry lips, paresthesia, and suture dehiscence. There was no statistically significant association between complications and the technique used (p = 0.69). CONCLUSION: We concluded that vermilionectomy using the W-plasty technique shows better cosmetic results and similar complication rates.

Apresenta-se caso de epidermólise bolhosa adquirida inflamatória. Paciente do sexo masculino, 53 anos, há seis meses com erupção vesicobolhosa pruriginosa sobre base eritematosa no tronco, axilas e membros. O exame anatomopatológico mostrou bolha subepidérmica com neutrófilos. A imunofluorescência direta revelou depósitos lineares de IgG, IgA, IgM e C3 na zona da membrana basal, sendo a imunofluorescência indireta e o Salt Split Skin indireto negativos. Anticorpos antinucleares não reagentes. Houve melhora do quadro com prednisona e cicatrização de algumas lesões com formação de milia. Trata-se de apresentação rara de epidermólise bolhosa adquirida, com lesões iniciais predominantemente inflamatórias.

We report a case of an inflammatory variant of epidermolysis bullosa acquisita in a 53-year-old male, with itching blistering eruption on the trunk, armpits and limbs for six months. The skin biopsy specimen showed subepidermal blister with neutrophils. Direct immunofluorescence revealed linear depositions of IgG, IgA, IgM and C3 at the basement membrane; indirect immunofluorescence and salt Split Skin were negative. Antinuclear antibodies were also negative. Improvement of the blisters followed treatment with systemic corticotherapy and some lesions healed with milia. This is a rare presentation of epidermolysis bullosa acquisita, with inflammatory lesions at first.

A dermatose por IgA linear é doença bolhosa auto-imune subepidérmica rara, caracterizada pelo depósito linear de IgA na zona da membrana basal da epiderme. Nos relatos de gestação em pacientes com essa dermatose, nota-se sempre melhora do quadro clínico. Contrariando essas observações,é apresentado caso de dermatose por IgA linear induzida pela gestação, que demonstrou boa resposta terapêutica à dapsona e prednisona , sem complicações materno-fetais.

Linear IgA disease is a rare autoimmune subepidermal bullous disorder characterized by linear IgA deposits at the epidermal basement membrane zone. According to the literature, in patients who have linear IgA disease and become pregnant, the disease tends to improve. We report a case of linear IgA disease induced by pregnancy, successfully treated with dapsone and prednisone with no adverse effects observed in the patient and her newborns.