Dermatose Neutrofílica do Dorso das Mãos - a Propósito de Dois Casos Clínicos

O conceito de dermatoses neutrofílicas engloba diversas entidades que partilham aspetos clínicos e histológicos. Recentemente descrita, a dermatose neutrofílica do dorso das mãos é uma patologia com etiologia desconhecida que se assemelha, clinicamente, a uma variante localizada da Síndrome de Sweet com lesões predominantemente localizadas no dorso das mãos e com presença variável de sintomas gerais acompanhantes. Do ponto de vista histológico é caracterizada por um denso infiltrado inflamatório neutrofílico, sendo variável a existência de achados de vasculite. Os autores apresentam dois doentes com dermatose neutrofílica do dorso das mãos e discutem os aspetos clínicos, laboratoriais, histopatológicos e a as opções terapêuticas desta entidade. A dermatose neutrofílica do dorso das mãos é um conceito em evolução, que partilha diversos aspetos comuns com outras dermatoses neutrofílicas, sugerindo um espetro continuo deste grupo de doenças.

Anti-Tumor Necrosis Factor Alpha-Induced Drug Eruptions: One Patient, More Than a Pattern

Background: Tumor necrosis factor alpha (TNFα) antagonists are effective in treating several immune-inflammatory diseases, including psoriasis and inflammatory bowel disease. The paradoxical and unpredictable induction of psoriasis and psoriasiform skin lesions is a recognized adverse event, although of unclear aetiology. However, histological analysis of these eruptions remains insufficient, yet suggesting that some might constitute a new pattern of adverse drug reaction, rather than true psoriasis. Case report: The authors report the case of a 43-year-old woman with severe recalcitrant Crohn disease who started treatment with infliximab. There was also a personal history of mild plaque psoriasis without clinical expression for the past eight years. She developed a heterogeneous cutaneous eruption of psoriasiform morphology with pustules and crusts after the third infliximab infusion. The histopathological diagnosis was of a Sweet-like dermatosis. The patient was successfully treated with cyclosporine in association with both topical corticosteroid and vitamin D3 analogue. Three weeks after switching to adalimumab a new psoriasiform eruption was observed, histologically compatible with a psoriasiform drug eruption. Despite this, and considering the beneficial effect on the inflammatory bowel disease, it was decided to maintain treatment with adalimumab and to treat through with topicals, with progressive control of skin disease. Discussion: Not much is known about the pathogenesis of psoriasiform eruptions induced by biological therapies, but genetic predisposition and Koebner phenomenon may contribute to it. Histopathology can add new facets to the comprehension of psoriasiform reactions. In fact, histopathologic patterns of such skin lesions appear to be varied, in a clear asymmetry with clinical findings. Conclusion: The sequential identification in the same patient of two clinical and histopathologic patterns of drug reaction to TNFα antagonists is rare. Additionally, to the authors’ knowledge, there is only one other description in literature of a TNFα antagonist-induced Sweet-like dermatosis, emphasizing the singularity of this case report.

Mastocytosis: a Rare Case of Anaphylaxis in Paediatric Age and Literature Review

The term “mastocytosis” denotes a heterogeneous group of disorders characterised by abnormal growth and accumulation of mast cells (MC) in one or more organ systems. Symptoms result from MC chemical mediator’s release, pathologic infiltration of neoplastic MC in tissues or both. Multiple molecular, genetic and chromosomal defects seem to contribute to an autonomous growth, but somatic c-kit D816V mutation is more frequently encountered, especially in systemic disease. We present a literature review of mastocytosis and a rare case report of an 18 month-old-girl with a bullous dermatosis, respiratory distress and anaphylaxis, as clinical manifestations of mastocytosis. The developments of accepted classification systems and novel useful markers allowed a re-evaluation and updating of the classification of mastocytosis. In paediatric age cutaneous forms of disease prevail and may regress spontaneously. SM is more frequently diagnosed in adults and is a persistent(clonal) disease of bone marrow. The clinical course in these patients is variable.Today diagnostic criteria for each disease variant are reasonably well defined. There are, however, peculiarities, namely in paediatric age, that makes the diagnostic approach difficult. Systemic disease may pose differential diagnostic problems resulting from multiple organ systems involvement. Coversly, the “unexplained” appearance of those symptoms with no skin lesions should raise the suspicion of MC disease. This case is reported in order to stress the clinical severity and difficult diagnostic approach that paediatric mastocytosis may assume.

Dermatose Febril. Um Diagnóstico a Não Esquecer

Apresenta-se o caso de um doente de 46 anos, com coinfecção VIH-1/VHC e cirrose hepática, internado por febre, prostração e astenia. Ao exame objetivo, observavam-se escassas pústulas necróticas em base eritematosa acantonadas aos dedos das mãos e pés com edema, dor e limitação funcional dos mesmos e da articulação tíbio-társica esquerda. Analiticamente, apenas elevação ligeira da enzimologia hepática e da PCR. Admitiram-se as hipóteses diagnósticas de endocardite, meningococcémia ou gonococcémia. Após isolamento de Neisseria gonorrhoeae em hemocultura, iniciou-se ceftriaxone 1g/dia EV com melhoria clínica. A positividade da PCR para N.gonorrhoeae no exsudado orofaríngeo confirmou o diagnóstico de gonococcémia disseminada de ponto de partida orofaríngeo. A gonorreia é uma infeção sexualmente transmitida causada pelo diplococo Gram negativo Neisseria gonorrhoeae. A gonococcémia disseminada na forma da síndrome clássica “dermatite-artrite” acompanha apenas 1-2% das infeções mucosas. A gonorreia orofaríngea é geralmente assintomática em homens e mulheres, constituindo provavelmente um reservatório importante do agente. O aumento da incidência de gonorreia torna este caso pertinente na prática clínica actual do dermatologista.

Porokeratosis Ptychotropica of the Scrotum: Dermoscopic Evaluation of an Atypical Presentation

Porokeratosis 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.