Browsing by Author "Fidalgo, A"
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- Cutaneous Leiomyomatosis in a Mother and DaughterPublication . Lencastre, A; Cabete, J; Gonçalves, R; João, A; Fidalgo, AA 34-year-old woman with no known medical history was evaluated for multiple painful brown nodules and papules on the anterior aspect of the trunk. She mentioned a history of similar cutaneous findings on her mother. Biopsies of three lesions revealed piloleiomyomata. Renal and adrenal ultrasound revealed an isolated simple cortical cyst, and pelvic and endovaginal ultrasound revealed two uterine myomata. The clinical diagnosis of hereditary leiomyomatosis and renal cell cancer was corroborated by the identification of a heterozygous variant on exon 5 of the fumarate hydratase gene (c.578C>T p.T193I). Identification of the tumor piloleiomyoma should alert the dermatologist to this rare genodermatosis, which is associated with an increased risk of renal cell tumors, demanding multidisciplinary follow-up, and personal and family counseling.
- Cutaneous Leukemic Infiltration Following Varicella - a Case of Wolf's Isotopic ResponsePublication . Brasileiro, A; Lencastre, A; João, A; Fidalgo, AWolf's isotopic response designates the appearance of two subsequent unrelated dermatoses in the same anatomic location. We report the case of a 51-year-old man with a medical history of chronic lymphocytic leukemia without known extra-hematopoietic involvement. The patient developed a disseminated papulo-vesiculous eruption, diagnosed as varicella. Few days after recovering, an erythematous and violaceous papular dermatosis with histopathological examination compatible with leukemic infiltration appeared on the scars of previous herpetic lesions. Complete remission was obtained under systemic corticotherapy, without cutaneous recurrence or blastic transformation. Wolf's isotopic response is attributed to a localized immunologic imbalance following a certain stimulus. In this patient, herpetic infection acted as a local spur for inaugural cutaneous leukemic infiltration, with no impact on the prognosis for the underlying disease.
- Do Steroid Hormones Have an Important Role on Cutaneous Lupus Physiopathology?Publication . Brasileiro, A; Campos, S; Fidalgo, A
- Intraepidermal Epidermotropic Metastatic Melanoma: a Clinical and Histopathological Mimicker of Melanoma in Situ Occurring in MultiplicityPublication . Lestre, S; João, A; Ponte, P; Peixoto, A; Vieira, J; Teixeira, MR; Fidalgo, AThe distinction between primary melanoma and melanoma metastatic to the skin has major prognostic implications. We report a case of a 67-year-old male with a diagnosis of a superficial spreading melanoma (stage IB) rendered 6 years earlier who presented clinically with an atypical nevus on his left thigh. Histopathological examination showed an intraepidermal melanocytic proliferation that was interpreted as melanoma in situ. Subsequently, 45 additional pigmented macules appeared in crops over a 9-month period. Clinically and dermoscopically, these lesions were extremely polymorphic. Histopathological findings were compatible with melanoma in situ, as each lesion consisted of a wholly intraepidermal proliferation of markedly atypical melanocytes arranged singly and in nests. A complete gastrointestinal study showed multiple pigmented metastatic lesions throughout the stomach and small bowel, which supported a diagnosis of metastatic melanoma with gastrointestinal and epidermotropic skin involvement. Monosomy of chromosome 9 and a BRAF V600E mutation were detected in the primary tumor sample and in macro-dissected secondary lesions. No CDKN2A or CDK4 germline mutations were found. Intraepidermal epidermotropic metastases of melanoma have been rarely described in literature. In this case, histopathology alone was insufficient to distinguish metastatic melanoma from multiple in situ melanomas. The recognition of epidermotropic metastases should be based on the correlation between clinical, dermoscopic, histopathological and molecular findings.
- Intravenous Immunoglobulin for the Treatment of Quetiapin-Induced Bullous Pemphigoid in an HIV-Infected PatientPublication . Cabete, J; Santos, G; Páris, F; Serrão, V; Fidalgo, AThe authors present the case of a 48-year-old woman with HIV-associated dementia treated with antiretroviral therapy and psychoactive drugs, to whom bullous pemphigoid(BP) was diagnosed. Given incomplete response to corticotherapy, and azathioprine-induced bicytopenia,intravenous immunoglobulin(IVIG) was initiated. Despite transient disease control, recurrent flares suggested a persistent triggering factor. Specifically, quetiapin was implicated and discontinued with an immediate clinical response. Inadvertent re-challenge with olanzapine(a related drug)led to a new eruption, confirming drug-induced BP (DIBP). A total of six IVIG cycles were completed, without severe side effects to report, namely HIV disease progression. HIV-related autoimmune bullous diseases are rare. Treatment of severe drug eruptions is primarily based on immunossupressive drugs, raising concerns regarding additional immunossupression. This case suggests IVIG as a valuable option for the treatment of BP in HIV patients. In addition, quetiapin should be added to the list of neuroleptics previously linked to DIBP.
- Pênfigo EritematosoPublication . Baptista, J; Páris, F; Fidalgo, A; Cochito, M; Ferreira, APênfigo Eritematoso (PE), igualmente conhecido como síndroma de Senear-Usher, foi descrito pela primeira vez em 1926 por estes autores. É uma doença rara, de natureza auto-imune, que combina características clínicas e serológicas de pênfigo e lupus eritematoso. Em conjunto com o pênfigo foliáceo, pertence ao grupo dos pênfigos superficiais, que se caracterizam por acantólise e formação de bolhas nas camadas subcórneas da epiderme; distingue--se deste último pela distribuição das lesões, localizadas às áreas seborreicas do tronco e face, e pela deposição relativamente frequente de imunoglobulinas e complemento na junção dermo-epidérmica, para além dos espaços intercelulares das camadas subcórneas da epiderme. Apresenta-se o caso de uma doente do sexo feminino, 26 anos, caucasiana, com erosões e placas eritematocrostosas localizadas predominantemente nas áreas seborreicas da face e regiões pré-esternal e interescapular do tronco; na face as lesões assumiam uma distribuição típica em “asa de borboleta”, com agravamento durante a fotoexposição. O exame anatomopatológico e a imunofluorescência directa das lesões confirmaram o diagnóstico clínico de PE. Foi feito tratamento com corticoterapia sistémica, hidroxicloroquina e tacrolimus tópico, com evolução clínica rapidamente favorável.
- Phototherapy in Childhood: a 17-Year Retrospective Study Regarding Effectiveness and SafetyPublication . Brasileiro, A; Campos, S; Páris, F; Fidalgo, A; Apetato, MIntroduction: Skin diseases in paediatric age are often distressing conditions with significant impact in children’s psychosocial development. Additionally, systemic therapeutic options are often limited in childhood, due to its potential toxicity in this vulnerable group. Phototherapy is therefore an endorsed option for photo-responsive dermatological conditions. Objective and Methods:This observational retrospective study aims to access efficacy and safety of Phototherapy in our paediatric population. Relevant clinical data from 1996 to present concerning patients aged 18 years or less was collected. Results: 78 patients were included, of which64,1%was female. Mean age was 12,9 years (range 2-18). Distribution according to diagnosis was:47,4%psoriasis, 34,6% alopecia areata, 9,0% vitiligo, 9,0% other diagnosis. Mean number of cycles was 1,5 (range 1-7), with an average of 16,3 treatments per cycle and mean cumulative dose 134 J/cm2. 70,5% was treated with one single cycle. Topic and systemic PUVA were the first choice in 37,2% and 39,7%, respectively, while UVB TL01 and broadband UVB were used in 11,5% each. On the first cycle 67,5% improved, 14,3% showed no sustained clinical response and 19,5% were lost to follow-up. Psoriasis patients had the best response rates (81,8%), followed by alopecia areata (59,3%). Side effects occurred in 21%, being erythema the most common (12%). None led to therapeutic interruption. Discussion: Phototherapy is a safe and effective option in childhood, yet the withdraw rate might be an important limitation.
- Porokeratosis Ptychotropica of the Scrotum: Dermoscopic Evaluation of an Atypical PresentationPublication . Cabete, J; Fidalgo, A; Lencastre, A; Diamantino, F; João, APorokeratosis ptychotropica is a rare variant of porokeratosis that is classically located on the gluteal and perianal regions, seldom extending to the genitalia. The authors report an atypical presentation of porokeratosis ptychotropica and discuss the use of dermoscopy in evaluating this dermatosis. Dermoscopic findings, although not specific to this variant of porokeratosis, are helpful in the differential diagnosis of other genital disorders. Histopathology, through the visualization of multiple cornoid lamellae, prevails as the gold standard for the definite diagnosis of porokeratosis ptychotropica.
- Provas Epicutâneas sob Imunossupressão Sistémica - Contra-Indicação Absoluta?Publication . Brasileiro, A; Campos, S; Fidalgo, A; Lobo, LIntrodução: As provas epicutâneas (PE) são o exame complementar de diagnóstico indicado para avaliação de suspeita de dermite de contacto alérgica. Idealmente, devem ser realizadas sem que o doente se encontre sob imunossupressores. Existem contudo situações clínicas em que tal não é possível, não havendo informação disponível acerca de como realizar e valorizar os resultados das PE nestes doentes. O objectivo do presente trabalho é rever a literatura no que concerne à realização de PE sob imunossupressão iatrogénica. Material e Métodos: Revisão da literatura relevante para o tema publicada até Janeiro de 2015 e indexada à Medline. Resultados: De acordo com o reportado na literatura, foram realizadas PE em 77 doentes sob corticóide sistémico, 78 doentes sob ciclosporina (CyA), 6 sob azatioprina, 10 sob metotrexato (MTX), 4 sob micofenolato de mofetil (MMF), 11 sob fármacos anti-factor de necrose tumoral e 7 sob fármaco anti-IL-12/23. Foram ainda descritos 15 casos de realização de PE sob associação de imunossupressores. Verificaram-se reacções positivas em todos os grupos. Conclusão: O tratamento concomitante com imunossupressores não deve ser uma contra-indicação para realização de PE, estando descritas reacções positivas em doentes sob prednisolona, azatioprina, CyA, MTX, MMF, infliximab, etanercept, adalimumab e ustecinumab. Os resultados negativos ou duvidosos devem, contudo, ser interpretados de forma cautelosa.
- Scalp Porocarcinoma: a Rare DiagnosisPublication . Brasileiro, A; Santos, G; Fidalgo, A; João, AAdnexal skin tumours are rare conditions, and often clinically indistinguishable from other cutaneous neoplasms. Porocarcinoma, a sweat gland malignant tumour, is more commonly found on extremities. Few reports in other anatomic locations can be found in the literature, and those arising on the scalp are even scarcer. The authors report the case of an 84-year-old diabetic man, with a tumour on the left parietal region for 1 year, which histopathological features were consistent with porocarcinoma. The importance of histopathologic diagnosis is hereby emphasized by the more aggressive behaviour of this tumour, therefore requiring clinical actuation accordingly.