José Carlos Cardoso

Cutaneous sarcoidosis: a histopathological study

Backgroundu2002 Sarcoidosis is a granulomatous disease of uncertain aetiology in which the skin is frequently involved. Naked sarcoidal granulomas are the characteristic histological feature in specific lesions of sarcoidosis.

Allergic contact dermatitis from photobonded acrylic gel nails: a review of four cases

Artificial photobonded acrylic gel nails, used to improve the cosmetic appearance of natural nails, have gained popularity over recent years. They can cause allergic contact dermatitis both in occupational and in non-occupational settings. It affects not only the nail area but also the elsewhere on the hands, and occasionally involves the face, including the eyelids, mainly due to an occupational airborne dermatitis. Case Reports

Papilomatose cutânea florida e acantose nigricante maligna reveladoras de neoplasia gástrica

4 Abstract: This paper reports the case of a 57-year-old, previously healthy male with no systemic symp- toms who over a short period of time developed multiple wart-like lesions on his trunk, limbs and face, typical lesions of acanthosis nigricans in the major body folds and tripe palms. Diagnostic tests revealed a metastatic gastric adenocarcinoma. Despite the implementation of therapy, which had a transient effect on the tumor and skin lesions, the patient died in 14 months. The association of these three para- neoplastic dermatoses (florid cutaneous papillomatosis, acanthosis nigricans maligna and tripe palms) in the same patient, apparently with a common pathogenic mechanism, is noteworthy. Keywords: Acanthosis nigricans, paraneoplastic syndromes; Stomach neoplasms Resumo: Apresenta-se o caso clinico de um doente de 57 anos, previamente saudavel, sem sintoma- tologia sistemica, que, num curto intervalo de tempo, desenvolve multiplas lesoes semelhantes a verru- gas virais no tronco, membros e face, lesoes tipicas de acantose nigricante nas grandes pregas e uma queratodermia difusa palmar com paquidermatoglifia. Os exames complementares de diagnostico rev- elaram uma neoplasia gastrica metastizada. Apesar da instituicao da terapeutica, com efeito transitorio na neoplasia e nas lesoes cutâneas, o doente viria a falecer em 14 meses. Salientamos a associacao destas tres dermatoses paraneoplasicas num mesmo paciente: papilomatose cutânea florida, acantose nigricante maligna e tripe palms que parecem ter um mecanismo patogenico comum. Palavras-chave: Acantose nigricans; Neoplasias gastricas; Sindromes paraneoplasicas

Pyoderma gangrenosum—a novel approach?

SummaryPyoderma gangrenosum (PG) represents axa0rare skin disorder, with several clinical variants and still not fully understood ethiopathogenesis. Often associated with inflammatory or neoplastic disease, PG is nowadays considered an inflammatory neutrophilic disease with common underlying morbidity. Modern treatment options are oriented towards key mechanisms underlying the pathogenesis of the disease, namely inflammatory mediators, and seem to be the most effective treatment currently available. Although promising, the results are not invariable and these treatments are sometimes surrounded by controversy, as recent studies have reported cases that are refractory to therapy with biological agents. It is possible that refractoriness to the use of biological agents as monotherapy stems from the fact that axa0single agent is not able to affect the entire inflammatory cascade, or to simultaneously influence all of its levels. Based on the pathogenesis of inflammation, we can suggest that an ideal targeted therapy should be able to induce the following changes: 1)xa0reduction of the secretion of interleukin (IL)-1a/b from the inflammasome with subsequent blocking of its biological effect (by therapy with IL-1 receptor antagonists); 2)xa0blocking of the activation of the secreted procytokines in their active form (by therapy with caspase-1 inhibitors; 3)xa0blocking of the effect of the already released active cytokines (by therapy with tumour necrosis factor alpha, TNF-α, inhibitors); 4)xa0blocking of the effector action of the cytokines on the target intracellular molecules (by therapy with kinase inhibitors). The specific therapy should aim to attack more than one link in the inflammatory cascade, in order to achieve maximum therapeutic effectiveness. Most surely, this could be achieved with combined therapy with different groups of biological agents (for example axa0combined therapy with IL-1 receptor antagonist and axa0TNF-α inhibitor). Currently, no data in the literature exist to support this statement, and there are no safety data relating to such approaches. We focus this review on the novel etiopathogenetic concepts of PG and the future therapeutic approaches based on blocking different levels of the inflammatory cascade, which seems to be the most promising weapon in the target-oriented treatment options.ZusammenfassungDas Pyoderma gangraenosum (PG) ist eine seltene Hauterkrankung mit verschiedenen klinischen Subtypen und einer noch immer nicht komplett verstandenen Ätiopathogenese. Das PG wird als entzündliche neutrophile Dermatose verstanden, die oftmals eine Assoziation zu anderen entzündlichen oder neoplastischen Erkrankungen zeigt. Moderne Behandlungsansätze orientieren sich an den Schlüsselpositionen der zugrunde liegenden Pathogenese, insbesondere den inflammatorischen Mediatorsubstanzen. Sie verkörpern die derzeit effektivsten Therapieoptionen. Obwohl derxa0Ansatz durchaus erfolgversprechend ist, sind die klinischen Resultate nicht konstant. Der Umstand sog. therapierefraktärer Fälle gegenüber einer zielgerichteten Therapie wird kontrovers diskutiert. Dieses Therapieversagen könnte jedoch auch daher rühren, dass eine Monotherapie mit Biologika nicht in der Lage ist, die gesamte Entzündungskaskade zu beeinflussen bzw. gleichermaßen simultan zu kontrollieren. Auf der Entzündungspathogenese basierend schlagen die Autoren für eine ideale Therapie folgende Eckpunkte vor: 1)xa0Verminderung der IL-1a/b-Sekretion des Inflammasoms mit nachfolgender Blockade ihrer biologischen Wirkung (durch Einsatz von IL-1-Rezeptorantagonisten); 2)xa0Blockade der Aktivierung sezernierter Prozytokine in ihrer aktiven Form (durch Einsatz von Caspase-1-Inhibitoren; 3)xa0Hemmung der bereits sezernierten Zytokine (durch TNF-α-Inhibitoren); 4)xa0Blockade der Effektoraktion der Zytokine auf entsprechende intrazelluläre Moleküle (mittels Kinaseinhibitoren). Die spezifische Therapie sollte an mehreren Schnittstellen der Entzündungskaskade angreifen, um eine maximale Wirkung zu entfalten. Aller Wahrscheinlichkeit nach lässt sich dieses Ziel nur durch Kombination verschiedener Biologika errreichen (z.u2009B. eine Kombination von IL-1-Rezeptorantagonist und TNF-α-Inhibitor). Allerdings existieren derzeit noch keine harten wissenschaftlichen Daten, die dieses Konzept unterstützen. Ein weiterer ungeklärter Aspekt ist die Patientensicherheit. Die vorliegende Übersicht fokussiert auf neue ätiopathogenetische Konzepte zumxa0PG und daraus abzuleitende zukünftige Behandlungsansätze, die auf dem Prinzip basieren, die Entzündungskaskade auf verschiedenen Ebenen zu unterbrechen. Dies scheint der erfolgversprechendste Ansatz einer zielgerichteten Therapie zu sein.

Aquagenic keratoderma. Two new case reports and a new hypothesis

Aquagenic keratoderma has been described as a transient condition affecting predominantly young females and defined clinically by the appearance of palmar hyper-wrinkling accentuated after immersion in water. We present two new cases with aquagenic palmoplantar acrokeratoderma – a child and a young male. A significant clinical improvement was achieved after topical treatment with aluminum salts. Aquagenic palmar keratoderma may be a clue to cystic fibrosis in adolescents and young adults. We developed a new hypothesis on its pathogenesis.

Histiocitomas fibrosos: revisão histopatológica de 95 casos

Fibrous histiocytoma (FH) is a heterogeneous tumor composed of fibroblasts, histiocytes, and blood vessels. We conducted a retrospective histopathologic analysis of 95 biopsies, performed over the last 3.5 years, of fibrous histiocytomas to analyze the location, delimitation, epithelial changes, induction of folliculo-sebaceous structures, cellularity, vascularity, collagen pattern, and types of composite cells of the FH. In the majority of the biopsies, we confirmed the classical histopathologic features of fibrous histiocytomas. The presence of lymphoid nodules, mast cells, and sparse infiltrate of inflammatory cells was an interesting finding observed in our study.

Overlap between maculopapular exanthema and drug reaction with eosinophilia and systemic symptoms among cutaneous adverse drug reactions in a dermatology ward.

Inpatients with cutaneous adverse drug reactions (CADR) with overlapping features between maculopapular exanthema (MPE) and drug reaction with eosinophilia and systemic symptoms (DRESS) were examined.

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.

Merkelzellkarzinom: eine Überprüfung und Aktualisierung über die Pathogenese, Diagnose und Therapieansätze

Merkel cell carcinoma (MCC) or primary cutaneous neuroendocrine carcinoma is a relatively uncommon form of skin cancer. It is an aggressive neoplasm with high tendency for local recurrence and lymph node and distant metastasis, whose incidence has been rising in the last 2 decades.A novel polyomavirus (MCPyV) has been found in a significant proportion of MCCs, and this finding significantly contributed to the understanding of its pathogenesis.It is fairly consensual at the current state of know-ledge that appropriate staging, including sentinel lymph node biopsy, is very important in order to plan adequate treatment. Treatment includes aggressive surgery of the primary tumour and lymph node basin, commonly combined with adjuvant radiotherapy. Chemotherapy is usually reserved for distant metastasis.In the present article, the authors review the current knowledge about MCC with special emphasis on the new pathogenetic findings and current recommendations regarding management.SummaryMerkel cell carcinoma (MCC) or primary cutaneous neuroendocrine carcinoma is a relatively uncommon form of skin cancer. It is an aggressive neoplasm with high tendency for local recurrence and lymph node and distant metastasis, whose incidence has been rising in the last 2 decades.A novel polyomavirus (MCPyV) has been found in a significant proportion of MCCs, and this finding significantly contributed to the understanding of its pathogenesis.It is fairly consensual at the current state of know-ledge that appropriate staging, including sentinel lymph node biopsy, is very important in order to plan adequate treatment. Treatment includes aggressive surgery of the primary tumour and lymph node basin, commonly combined with adjuvant radiotherapy. Chemotherapy is usually reserved for distant metastasis.In the present article, the authors review the current knowledge about MCC with special emphasis on the new pathogenetic findings and current recommendations regarding management.ZusammenfassungDas Merkelzellkarzinom (MCC) oder primäres kutanes neuroendokrines Karzinom ist ein relativ seltener Hautkrebs. Es handelt sich um eine aggressive Neubildung mit einer Neigung zu lokalen Rezidiven, Lymphknoten- und Fernmetastasen. Seine Inzidenz ist in den letzten zwei Jahrzehnten angestiegen.Die Entdeckung eines neuen Polyomavirus (MCPyV) bei zahlreichen MCC hat unser Pathogeneseverständnis deutlich beeinflusst.Es herrscht Konsens darüber, dass ein entsprechendes Staging einschließlich der Sentinel-Lymphknotenbiopsie für eine adäquate Therapieplanung unbedingte Vorraussetzung ist. In der Behandlung kommen die aggressive Tumorchirurgie des primären MCC und der regionalen Lymphknotenstation (bei positivem Sentinel) in Kombination mit einer adjuvanten Strahlentherapie zum Einsatz. Die Chemotherapie ist für gewöhnlich dem Stadium der Fernmetastasierung vorbehalten und rein palliativer Natur.In der vorliegenden Arbeit geben wir eine Übersicht zur Kenntnis des MCC mit Betonung auf die neueren Befunde zur Pathogenese sowie zu aktuellen therapeutischen Empfehlungen.

Immediate ex-vivo dermoscopy of a nail bed biopsy specimen – a useful procedure for melanonychia

bulocystic squamous cell carcinoma as a differential diagnosis. Infundibulocystic squamous cell carcinoma which is known to have the distinctive histopathological features of numerous microor dilated infundibular cysts with minimal cytological atypia that infiltrate and penetrate deep into the underlying tissue. In our case, the tumour cells without evident nuclear atypia did not penetrate in the deep dermis and clinical manifestation of which present symmetrical, exophytic and central erosion. Furthermore, long-standing history indicated the benign lesion, so we excluded infundibulocystic squamous cell carcinoma. The dermoscopic features of millia-like cysts often resemble those of seborrhoeic keratosis; however, in our case, typical dermoscopic features of seborrhoeic keratosis, such as comedolike openings, fingerprint-like structures and brain-like appearances, were not observed. We regarded the lack of brownish background as a unique characteristic. We think these features, especially the multiple millia-like cysts on a background of reddish plaques, might be helpful in the clinical diagnosis of keratoacanthoma en plaque. In conclusion, this is the first report to describe the dermoscopic features of keratoacanthoma en plaque with infundibulocystic hyperplasia. When we encounter reddish plaques with ‘millia-like cysts on an erythematous background’ in dermoscopy, we must consider keratoacanthoma en plaque with infundibulocystic hyperplasia as a differential diagnosis.