Miguel Gouveia

Interstitial Granulomatous Dermatitis: A Clinicopathological Study.

Introduction:Interstitial granulomatous dermatitis (IGD) is an uncommon granulomatous dermatitis occurring in the setting of highly reactive immune states, with a polymorphic clinical presentation. Because there is overlap with other entities [namely palisading neutrophilic granulomatous dermatitis (PNGD)], controversy exists regarding its classification. Objective:To understand if there are features allowing clear-cut distinction between IGD and PNGD. Material and Methods:Retrospective analysis of 10 cases previously diagnosed as IGD or PNGD, from 2000 to 2013, with review of the histopathologic findings and clinical correlation. Results:Six females and 4 males presented mostly with erythematous papules/nodules (n = 7) but also with erythematous annular plaques (n = 3). In 2 patients, the lesions coexisted. They were mostly distributed symmetrically on the limbs. Associated disease was observed in 6 patients. Regarding histopathology, an inflammatory infiltrate occupying the superficial and mid dermis was present in 40% of cases, with an interstitial component in all biopsies and a palisaded arrangement in 60%. Neutrophils and mononuclear cells were present in all cases in varying proportions. Necrobiosis was found in 70%, and leukocytoclasia was observed in 80% of biopsies. Conclusions:Coexistence of the interstitial and palisaded inflammatory patterns occurred in 90% of cases, with no correlation between tissue neutrophilia and the predominant pattern of the infiltrate. There was also no clear-cut correlation between the infiltrate pattern and semiologic aspect of the lesions. Therefore, the features described in our study suggest that IGD and PNGD belong to the same clinicopathological spectrum.

Clinical characterization and long-term follow-up of Schnitzler syndrome

Schnitzler syndrome (SchS) is an acquired autoinflammatory disease characterized by chronic urticarial rash in association with monoclonal gammopathy. Patients may progress to lymphoproliferative disorders, but the associated factors and exact risk of progression are still not well defined.

Histological variability and the importance of clinicopathological correlation in cutaneous Rosai-Dorfman disease

Rosai-Dorfman disease is a benign histiocytic proliferative disorder of unknown etiology. The disease mainly affects lymph node tissue, although it is rarely confined to the skin. Here, we describe a 53-year-old woman with purely cutaneous Rosai-Dorfman disease. The patient presented with a large pigmented plaque on her left leg, and sparse erythematous papules on her face and arms. A complete clinical response was achieved with thalidomide, followed by recurrence at the initial site one year later. The histological examination displayed the typical features of Rosai-Dorfman disease in the recent lesions but not in the older lesions. In the setting of no lymphadenopathy, the histopathological features of Rosai-Dorfman disease are commonly misinterpreted. Therefore, awareness of the histological aspects present at different stages, not always featuring the hallmark microscopic signs of Rosai-Dorfman disease, is particularly important for a correct diagnosis of this rare disorder.

Childhood hypopigmented mycosis fungoides: a commonly delayed diagnosis.

Primary cutaneous lymphomas (PCLs) are exceedingly rare in children and adolescents, with mycosis fungoides (MF) being the most frequent PCL diagnosed in childhood. There are numerous unusual clinical variants of MF, including the hypopigmented type form (HMF). HMF is exceptional overall, but comparatively common among children. We present an 8-year-old boy with a 3-year history of progressive, generalised, scaly, hypopigmented round patches and few erythematous papules. He was first diagnosed with pityriasis alba (PA), and moisturisers were prescribed with no improvement. Skin biopsy showed typical features of MF, and the patient was successfully treated with narrowband ultraviolet B. HMF may simulate atopic dermatitis, PA, pityriasis lichenoides, tinea versicolour, vitiligo, postinflammatory hypopigmentation or leprosy. Therefore, persistent and unusual hypopigmented lesions should be biopsied to rule out this rare variant of MF.

Do you know this syndrome? Dyspigmentation along the Blaschko lines caused by trisomy 7 mosaicism

Dyspigmentation along the Blaschko lines is strongly suggestive of a mosaic skin disorder. We report a 9-year-old male patient who presented with swirls and streaks of both hypo and hyperpigmentation involving the entire body. Additionally, he had hypertrichosis, musculoskeletal and minor neurodevelopment abnormalities but no intellectual disability. Cultured fibroblast displayed trisomy 7 mosaicism, which can explain this pigmentary phenotype. Widespread dyspigmentation associated with involvement of other organs should prompt systemic examination to detect additional anomalies and genetic evaluation should be considered, even with normal fetal karyotype.

Non-melanoma skin cancer in Portuguese kidney transplant recipients - incidence and risk factors.

Background Cancer is currently among the three leading causes of death after solid organ transplantation and its incidence is increasing. Non-melanoma skin cancer - squamous cell carcinoma and basal cell carcinoma - is the most common malignancy found in kidney transplant recipients (KTRs). The incidence of non-melanoma skin cancer in KTRs has not been extensively studied in Portugal. Objectives To determine the incidence of non-melanoma skin cancer in KTRs from the largest Portuguese kidney transplant unit; and to study risk factors for non-melanoma skin cancer. Methods Retrospective analysis of clinical records of KTRs referred for the first time for a dermatology consultation between 2004 and 2013. A case-control study was performed on KTRs with and without non-melanoma skin cancer. Results We included 288 KTRs with a median age at transplantation of 47 years, a male gender predominance (66%) and a median transplant duration of 3.67 years. One fourth (n=71) of KTRs developed 131 non-melanoma skin cancers, including 69 (53%) squamous cell carcinomas and 62 (47%) basal cell carcinomas (ratio squamous cell carcinoma: basal cell carcinoma 1.11), with a mean of 1.85 neoplasms per patient. Forty percent of invasive squamous cell carcinomas involved at least two clinical or histological high-risk features. The following factors were associated with a higher risk of non-melanoma skin cancer: an older age at transplantation and at the first consultation, a longer transplant duration and the presence of actinic keratosis. KTRs treated with azathioprine were 2.85 times more likely to develop non-melanoma skin cancer (p=0.01). Conclusion Non-melanoma skin cancer was a common reason for dermatology consultation in Portuguese KTRs. It is imperative for KTRs to have access to specialized dermatology consultation for early referral and treatment of skin malignancies.

Overlap between maculopapular exanthema and DRESS among cutaneous adverse drug reactions in a dermatology ward (2008-2012)

Background Immune-mediated cutaneous adverse drug reactions (CADR) present under different clinical patterns, some different from the main phenotypes of CADR. Our objective is to characterize manifestations and culprit drugs in CADR that required hospitalization, particularly exanthema associated with few systemic symptoms without fulfilling the European DRESS criteria (MP/DR).

FIBROXANTOMA ATÍPICO – TUMOR RARO SOBRE CICATRIZ DE QUEIMADURA

O fibroxantoma atipico (FXA) e um cancro cutâneo relativamente raro que surge mais frequentemente como lesao unica, de crescimento rapido, em areas fotoexpostas de individuos idosos. Apesar de apresentar caracteristicas de agressividade a nivel histologico, nomeadamente pleomorfismo e elevado indice mitotico, o prognostico tende a ser favoravel. Apresentamos o caso de uma mulher de 62 anos, com area de alopecia cicatricial no couro cabeludo secundaria a queimadura na infância, que desenvolve nesse local e em cerca de um ano, uma lesao tumoral vegetante. O exame anatomopatologico com imunohistoquimica da biopsia incisional foi sugestivo de FXA. Realizou-se a excisao radical com encerramento por duplo retalho de transposicao oposto, obtendo-se controlo local da neoplasia. Os mecanismos envolvidos no desenvolvimento do FXA nao estao bem esclarecidos, mas a exposicao cronica a radiacao ultravioleta sera um dos factores primordiais. Outros factores considerados sao a radioterapia e a imunossupressao. As cicatrizes de queimadura foram descritas apenas esporadicamente.

ANTIAGREGANTES E ANTICOAGULANTES EM CIRURGIA DERMATOLÓGICA – NORMAS DE ORIENTAÇÃO CLÍNICA

Introducao : A abordagem da terapeutica antitrombotica nao e consensual na cirurgia dermatologica. O dermatologista tem de ponderar entre o risco hemorragico e o risco trombotico quando decide do manuseio destes farmacos no periodo perioperatorio. Material e Metodos : Os autores reviram a literatura disponivel com o intuito de elaborar normas de orientacao clinica para a abordagem destes doentes. Resultados : Ha poucos estudos significativos publicados nesta area, e por vezes com diferentes abordagens e recomendacoes. O principal objetivo do cirurgiao dermatologico devera ser a otimizacao dos resultados cirurgicos sem esquecer os riscos tromboticos e hemorragicos inerentes ao doente e ao procedimento cirurgico. Conclusoes: Recomendamos a continuacao dos antitromboticos nos procedimentos dermatologicos com baixo risco hemorragico. Nos doentes com elevado risco tromboembolico recomendamos a continuacao do farmaco antitrombotico nos procedimentos de baixo risco hemorragico, e a ponderacao da sua suspensao com substituicao por heparina de baixo peso molecular nos procedimentos de elevado risco hemorragico. No entanto, estas recomendacoes globais nao se devem sobrepor a uma abordagem individualizada a situacao clinica pontual de cada doente.

Multiple minute digitate hyperkeratosis - a peculiar entity

Multiple minute digitate hyperkeratosis is a rare, non-follicular dermatosis, with fewer than 30 cases described worldwide. It can be either acquired or inherited in an autosomal dominant pattern. We describe the case of an 83-year old patient with life-long, multiple, digitate, milimetric lesions, and a positive family history for the same dermatosis.