Glória Velho

Poroceratose superficial disseminada num doente com colangiocarcinoma: manifestação paraneoplásica?

Porokeratosis refers to a group of hereditary or acquired disorders of epidermal keratinization and is characterized histologically by the presence of a cornoid lamella. The clinical variant referred to as disseminated superficial porokeratosis has been described in the literature in association with immunosuppressive conditions that include organ transplant, infections and immunosuppressive treatments. The association of disseminated superficial porokeratosis with solid organ malignancies has seldom been described, only 5 such cases having been published. The present report refers to a patient with lesions of disseminated superficial porokeratosis of sudden onset shortly before diagnosis of a cholangiocarcinoma.

Scleromyxedema vs scleredema: a diagnostic challenge.

ejd.2011.1493 Auteur(s) : Iolanda Conde Fernandes iolanda.c.fernandes@iol.pt, Madalena Sanches, Gloria Velho, Ines Lobo, Rosario Alves, Manuela Selores Department of Dermatology, EPE – Hospital de Santo Antonio, Rua D. Manuel II, s/n, 4100 Porto, Portugal A 32-year-old Caucasian man presented with facial edema, associated with indurated and thickened skin of the trunk and superior extremities (figure 1A, B). The patient referred fever, myalgias, arthralgias (mainly in the hand joints which [...]

IgA pemphigus and pustular psoriasis: a possible relation

Auteur(s) : Aristoteles ROSMANINHO arisrosmaninho@gmail.com, Ana OLIVEIRA, Madalena SANCHES, Gloria VELHO, Isabel AMORIM, Manuela SELORES Department of Dermatology, Centro hospitalar do Porto-HSA, Rua D. Manuel II s/n°, 4099-001 Porto, Portugal A 68-year-old woman was referred to our clinic with a one month history of a generalized, pruritic, vesicopustular eruption. She had applied topical mometasone cream with no improvement. Her medical history was remarkable for a pustular psoriasis (confirmed histologically) [...]

Cutaneous sarcoid-like granulomas with alveolar hemorrhage and c-ANCA PR-3

A 28‐year‐old woman, employed as a leather factory worker, noted asymptomatic, well‐delimited plaques on both knees, 6 years ago. The plaques were violaceous with a smooth surface. One appeared over a post‐traumatic scar from childhood ( Fig. 1 ). Two years later, she began to complain of symptoms suggestive of polyarthritis, first of the small joints of the hands (proximal interphalanges) and then of the larger joints (wrists, elbows, and knees). She was diagnosed with rheumatoid arthritis and began treatment with nonsteroidal anti‐inflammatory drugs for 1 month without any change. Deflazacort, 12 mg/day, and hydroxychloroquine, 400 mg/day, were administered for 3 months, with improvement of her articular complaints, but not her skin lesions.

Unilateral nevoid telangiectasia--report of two cases.

Unilateral nevoid telangiectasia – report of two cases Unilateral nevoid telangiectasia (UNT) is a rare condition characterized by telangiectasias distributed in a linear, unilateral pattern. The etiopathogenesis is not completely understood, although it is believed that high levels of estrogens are behind its development. It can be congenital or acquired. In these cases, it is related to hyperestrogenism. We report two cases of UNT in association with chronic liver disease without cirrhosis. The first refers to a 41-year-old woman who presented with multiple telangiectasias on her right upper thorax and shoulder (Fig. 1a) for five months. She had been submitted to a hepatic transplant four years before, after a fulminant hepatitis due to hepatitis B virus (HBV) infection, which was followed by treatment with lamivudine. Laboratory tests including complete blood cell count and biochemistry were unremarkable. The coagulation panel was within the normal ranges. Antibodies to HBV, which had been negative for the last four years, were positive, although DNA viral charge was within normal values. The hepatic ultrasound showed no cirrhosis. A skin biopsy was made revealing multiple dilated, thin-walled vessels in the papillary and upper reticular dermis (Fig. 1b,c). The second case refers to a 34-year-old man with hepatitis C virus (HCV) infection diagnosed three years before. The patient denied any prescribed or over-thecounter medication. He presented multiple telangiectasias over his left shoulder (Fig. 1d), which had appeared a few years before and had increased in number over the last few months. The laboratory tests were unremarkable, except for positive anti-HCV antibodies, although the RNA viral charge was within normal values. A skin biopsy showed dilated vessels in the superficial dermis. Unilateral nevoid telangiectasia is a vascular dermatosis that was first described in 1899 by Zeisler and Blaschko. It represents a unilateral and linear proliferation of superficial telangiectasias. It can be congenital or acquired. The congenital type is transmitted in an autosomal dominant pattern and is believed to be influenced by maternal estrogens. The acquired type frequently occurs in situations related to an increased estrogens’ production, such as puberty, pregnancy, the use of oral contraceptives, or chronic

A case of erythrokeratodermia variabilis with connexin 31 gene mutation (Cx31F137L).

before treatment with infliximab. The cutaneous nodules were decreased in size and number after TNF-a inhibition. The TNF-a inhibition of infliximab in this case possibly caused a partial reduction of the expression of MCP-1 in the cutaneous lesions, because the serum MCP-1 level was significantly decreased after the treatment. Taken together, MCP-1 and TNF-a might be more effective targets in the patients with MRH.

Lichen planus pemphigoides in a child.

ejd.2012.1764 Auteur(s) : Iolanda Conde Fernandes1 iolanda.c.fernandes@iol.pt, Teresa Pinto Almeida1, Isabel Mendes2, Gloria Cunha Velho1, Rosario Alves1, Manuela Selores1 1 Department of Dermatology Hospital de Santo Antonio Rua D. Manuel II, s/n 4099-001 Porto, Portugal 2 Department of Pediatrics Hospital de Faro, 8000-386 Faro, Portugal Bullous lesions, which are rarely seen in lichen planus, represent either bullous lichen planus (BLP) or lichen planus pemphigoides (LPP) [1]. Blisters arising [...]

Breast cancer: 2 case reports

Breast cancer is the most frequently diagnosed life-threatening cancer in women and the leading cause of cancer death among them worldwide. It includes a heterogeneous collection of diseases with various histologically defined subsets, clinical presentations, responses to treatment and outcomes. We describe 2 cases of female patients with ductal breast carcinoma. Dermatologists may have an important role in diagnosing such diseases.

MAJOR MULTIFORME ERYTHEMA WITH SERTRALINE

A sertralina e frequentemente utilizada para o tratamento de sindromes depressivos e ansiosos. Os efeitos secundarios sao geralmente transitorios e dependentes da dosagem. Descrevemos o caso clinico de uma mulher de 76 anos com sindrome depressivo e introducao recente da sertralina que recorreu ao servico de urgencia por febre e erupcao cutânea difusa com dois dias de evolucao. Ao exame objetivo observavam-se papulas eritematosas em alvo dispersas pelo corpo e exulceracoes da mucosa oral. Foi efetuada uma biopsia cutânea para exame histopatologico com diagnostico de eritema multiforme major secundario a sertralina. A doente suspendeu o farmaco em causa e iniciou prednisolona oral com resolucao do quadro clinico. Embora pouco frequentes, existem casos descritos de reacao cutânea grave associada a sertralina.

RITUXIMAB NO TRATAMENTO DE PÊNFIGO VULGAR REFRACTÁRIO

O penfigo vulgar e uma doenca bolhosa auto-imune rara, que atinge a pele e as mucosas. Geralmente tem um curso clinico severo, sendo necessario o recurso a terapeutica prolongada com corticoides sistemicos e outros farmacos imunossupressores, que podem conduzir a efeitos adversos graves. O rituximab e um anticorpo monoclo- nal quimerico dirigido ao antigenio CD20, expresso pelos linfocitos B. Recentemente, tem surgido alguns estudos que documentam o seu sucesso terapeutico no tratamento de penfigo refractario. Os autores descrevem dois casos clinicos de penfigo vulgar refractarios as terapeuticas convencionais, que foram tratados com rituximab, tendo atin- gido a remissao completa da doenca. A experiencia bem sucedida em relacao a estes dois casos clinicos reforca que o rituximab constitui uma opcao valiosa e segura na abordagem terapeutica do penfigo vulgar severo e refractario. PALAVRAS-CHAVE – Penfigo vulgar; Rituximab.O penfigo vulgar e uma doenca bolhosa auto-imune rara, que atinge a pele e as mucosas. Geralmente tem um curso clinico severo, sendo necessario o recurso a terapeutica prolongada com corticoides sistemicos e outros farmacos imunossupressores, que podem conduzir a efeitos adversos graves. O rituximab e um anticorpo monoclo- nal quimerico dirigido ao antigenio CD20, expresso pelos linfocitos B. Recentemente, tem surgido alguns estudos que documentam o seu sucesso terapeutico no tratamento de penfigo refractario. Os autores descrevem dois casos clinicos de penfigo vulgar refractarios as terapeuticas convencionais, que foram tratados com rituximab, tendo atin- gido a remissao completa da doenca. A experiencia bem sucedida em relacao a estes dois casos clinicos reforca que o rituximab constitui uma opcao valiosa e segura na abordagem terapeutica do penfigo vulgar severo e refractario. PALAVRAS-CHAVE – Penfigo vulgar; Rituximab.