RCAAP Repository

Introduction: Histiocytoses correspond to a heterogeneous group of disorders characterized by the proliferation and infiltration of histiocytes in tissues. For years, many of the histiocytoses where known by numerous names, reflecting the lack of understanding regarding their origin.Case report: A previously healthy, 4-month-old infant presented a cutaneous lesion of progressive growth on his nose since 1 month ago. The mother has a diagnosis of Sjögren syndrome. On examination, there was a firm well- -defined erythematous plaque with 30 mm of diameter and raised edges, on the bridge of the nose. At the left cheek, left thoracic region and right arm there were also small erythematous papules with 5 days of evolution. The analytical study showed elevated antinuclear antibodies and anti-SS-A. Histopathological examination confirmed the diagnosis of non-Langerhans cells histiocytosis. Progressively, more annular erythematous papules and plaques appeared scattered over the face, trunk and limbs with spontaneous resolution of the first lesion on the nose. About 2 months after the initial evaluation, all lesions had regressed spontaneously, with normalization of analytical study at one year-old.Conclusion: Many authors believe that the different subtypes of histiocytosis represent only different stages of the same disease. Immunophenotyping of histiocytosis does not always identify the subtype involved, making the occurrence of atypical cases frequent.

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Lichen aureus is a subtype of a chronic benign pigmented purpuric dermatosis of unknown etiology. It shows predilection for lower limbs and generally affects young adults and, less often, children. We report a case of lichen aureus with a unilateral presentation in a child.

A fasciíte eosinofílica é uma doença rara, caracterizada por edema simétrico doloroso com um progressivo espessamento da pele e tecidos moles, eosinofilia periférica, hipergamaglobulinemia policlonal e velocidade de hemossedimentação elevada. O diagnóstico pode ser feito por biópsia da pele até fáscia ou por ressonância nuclear magnética. Esta doença apresenta sintomas clínicos específicos, como o sinal do sulco que facilita o diagnóstico. Relatamos um caso de fasciíte eosinofílica em uma mulher de 48 anos de idade, com o endurecimento e espessamento da pele, que iniciou nas pernas, depois acometeu os braços e os antebraços e que apresentava o "sinal do sulco". A paciente foi tratada com sucesso com corticoterapia sistêmica e metotrexato.

Homenagem ao Professor Aureliano da Fonseca – XV Congresso Nacional de Dermatologia e Venereologia, 6 de Novembro de 2015, Porto

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Cutaneous pseudolymphoma refers to a heterogenous group of dermatosis which simulates cutaneous malignant lymphomas clinically and/or histopathologically. There are no diagnostic criteria to differentiate reactive from neoplastic infiltrates. Cutaneous pseudolymphoma can be divided into T- and B-cell variants depending on the predominant cell type in the infiltrate or classified according to the specific malignant lymphoma they simulate. This paper attempts to discuss the clinical and histological clues that are most relevant to the discrimination of lymphoma simulators from the true cutaneous lymphoma.

Sarcoidosis is an inflammatory multisystemic disease of unknown etiology, which is characterized by the formation of noncaseating granulomas in the organs involved, mainly lungs and intrathoracic lymph nodes. Skin involvement occurs in 25-35%; it is usually an early finding and it is an accessible and safe location for histopathological study. Cutaneous lesions may present with a variety of morphologies, including papules, nodules, plaques, and infiltrated scars. The diagnosis of sarcoidosis is supported by the recognition of compatible clinical, imagiological and histopathologic features, and the exclusion of other granulomatous disorders. Early diagnosis and systemic treatment could prevent future complications.

Introduction: There has been a significant advance in the understanding of the pathophysiology of bullous pemphigoid, particularly after demonstrating a pathogenic role for anti-BP180 IgE autoantibodies. Omalizumab is a monoclonal antibody that blocks free IgE and, in the last years, several cases of omalizumab-treated bullous pemphigoid have been published. This paper aims to clarify the pathogenic mechanisms of IgE autoantibodies in bullous pemphigoid and to discuss the clinical use of omalizumab based on the published clinical experience.Methods: Review of published articles in Medline/PubMed indexed journals using "bullous pemphigoid omalizumab" as search terms. Results: We review and discuss nine publications and related papers, when considered relevant by authors. In most cases, omalizumab seems to be an effective and safe drug in the treatment of bullous pemphigoid, being used more often as adjunct to other immunosuppressive agents. The role of biomarkers such as total IgE and eosinophil count in the selection of patients or treatment monitoring is still unknown.Conclusions: The use of omalizumab for the treatment of bullous pemphigoid is supported by the thoroughly studied pathogenicity of specific IgE autoantibodies. The published clinical experience is scarce, pointing omalizumab as a safe and effective option in bullous pemphigoid resistant to corticosteroids/immunosuppression. Because it is not an immunossupressive drug, omalizumab may be a valuable option in the treatment of bullous pemphigoid. Prospective randomized trials are warranted, particularly comparative studies with oral prednisolone in monotherapy.

Introduction: Wounds are a public health problem, with a strong impact on quality of life. They can be classified as chronic when they last more than six weeks. According to their origin, the most frequent are chronic pressure wounds, diabetic and vascular (arterial or venous) ulcers. A venous cause is found in about 80% of vascular wounds. This study aims to characterize chronic wounds from patients of ACeS PL area.Methods: This is a descriptive cross-sectional study, with an analytical component, using a convenience sample consisting of patients with chronic wounds, identified by nursing professionals, either at home or in primary care treatment rooms.Results: The prevalence of chronic wounds in the studied population is 0.84/1000, 1.1/1000 in men and 0.69/1000 in women (p < 0.05). Patients over 80 years of age have a prevalence of 5.68/1000, which is higher when compared to younger individuals (p < 0.05). Regarding their type, vascular wounds are the most common (36%) and of these, 77.7% have venous origin.Discussion and Conclusion: The prevalence of chronic wound is slightly lower than that found in other studies using the same methodology and is higher in men and in older ages. We found an excessive proportion of pressure ulcers of categories III and IV, verifying some nonconformity in their diagnosis and treatment. Hence, we suggest the creation of a wound consultancy and a multidisciplinary training group in the health service.

Introduction: Ultraviolet-B radiation is known to be effective and safe in childhood and adolescent psoriasis, but little has been published on the combined use of psoralen with ultraviolet-A radiation. The aim of this work is to assess the efficacy and short-term safety of systemic photochemotherapy in an adolescent population.Methods: Relevant clinical data on psoriatic patients aged 13 to 17 was retrospectively collected and analyzed. The sample population included twenty patients, eighteen with moderate to severe plaque-type psoriasis and two with guttate psoriasis.Results: Nine patients were treated with one single cycle and the remaining eleven with two cycles, in association with systemic therapies in 52%. Total improvement rate was 86.2%, with an average of 16.7 treatments per patient and a mean total cumulative dose of 174.0 J/cm2. The mean time for disease relapse was 4.3 months and two patients (10%) did not experience relapse during one-year follow-up. Side effects occurred in three patients (15%), namely nausea and erythema.Conclusions: Photochemotherapy showed to be highly effective in our adolescent population. Side effects were minor and none led to therapy discontinuation. Carcinogenic potential of ultraviolet-A radiation might be an important limitation. Further studies are warranted to access long-term safety of photochemotherapy.

Introduction: Sexually transmitted infections (STI) among men who have sex with men (MSM) have increased in recent years, particularly those caused by Neisseria gonorrhoeae (NG) and Chlamydia trachomatis (CT), both responsible for proctitis and anorectal infections.Methods: Retrospective analysis of the frequency of proctitis and NG and CT anorectal infection in 2420 MSM observed in Outpatient Clinic of STD of Lapa Health Center in Lisbon, Portugal, between January 2008 and December 2013. Sociodemographic, behavioral, clinical and laboratory data were assessed.Results: proctitis was identified in 1.6% (38 / 2420) of MSM. In 39.4% (15/38) of these patients, no infectious agent was identified. In 38 (16%) of the 238 MSM with NG infection, this agent had an anorectal location; this anatomical site was infected by CT in 23 (14.6%) of the 158 positive MSM. L2b genotype was identified in 7 of these 23 patients (30.4%). Anorectal infections were more frequent in immigrants, patients with a previous STI and with multiple sexual partners. The high frequency of MSM with anorectal infection that are asymptomatic or have no signs of proctitis should be emphasized: 55.3% (21/38) and 47.8% (11/23) of patients with NG and CT infection, respectively.Conclusions: The frequency of asymptomatic anorectal infection by CT and NG among MSM in Lapa Health Center is similar to that described in other countries and emphasizes the importance of screening these agents (including LGV strains) in anorectal swabs in MSM with receptive anal intercourse.

Lichen striatus is an inflammatory papular eruption that affects mainly children. It has an unknown etiology and a low prevalence. It arises in adulthood less frequently, and is characterized by abrupt onset of asymptomatic scaly papules in linear arrangement, sometimes following the Blaschko lines, usually unilateral and on the extremities. It is important to consider on the differential diagnosis other dermatoses that follow the Blaschko lines, such as linear lichen planus. We report an unusual case of lichen striatus with onset on the adulthood and located on left flank and on the left lumbar region. We discuss about the importance of dermatoscopy as an auxiliary tool on the diagnosis of lichen striatus.

Allergic contact dermatitis to acrylates and methacrylates is common in dentists and dental prosthetic professionals. The authors present a case of an atypical localization of allergic contact dermatitis to methacrylates in a dentist in order to alert to this reality and to implement methods to prevent sensitization to these allergens.

Introduction: The authors describe a case of annular elastolytic giant cell granuloma (AEGCG) associated with polycythaemia vera. Both diseases resolved with treatment with hydroxyurea.Case report: A 63-year-old man refers fatigue, nausea, right upper abdominal pain and marked weight loss for 4 months. During last month he presents multiple grouped erythematous skin papules and plaques in his back. He had a moderate increase of his spleen. The patient was diagnosed with polycythaemia vera (JAK 2 mutation positive in bone marrow). Skin biopsy revealed AEGCG. The patient was treated with hydroxyurea; there was normalization of hematologic changes and regression of skin lesions.Discussion: AECG is, in most cases, an isolated cutaneous disease but it can be found in association with other systemic diseases. Our patient is the first described with and association of AEGCG and polycythaemia vera. The regression of both diseases with hydroxyurea suggests a possible relationship.

During the last decades methicillin-resistant Staphylococcus aureus (MRSA) infections occurring within communities - previously documented only in nosocomial environments - increased, in relation with the development of bacterial resistance to alterations coded by mecA gene. We present the case report of a patient with recurrent furunculosis, confirmed by culture and antibiogram to be a methicillin-resistant Staphylococcus aureus (MRSA) infection community-associated. Therapy, instituted according to antibiogram, lead to complete clinic resolution.

Basal cell carcinoma represents the most common skin cancer and its incidence is increasing. Basal cell carcinoma is usually treated surgically or, as an alternative in selected cases, radiation therapy can be used either with curative or adjuvant intention. The designation “advanced basal cell carcinoma” is reserved for cases that cannot be treated by surgery and/or radiotherapy, or when these therapies would imply unacceptable morbidity. “Advanced basal cell carcinoma” includes as well the rare metastatic cases. Vismodegib is the first target drug for advanced basal cell carcinoma, inhibiting the hedgehog signaling pathway. The hedgehog pathway is the etiopathogenic base of Gorlin-Goltz syndrome, and is active in more than 90% of sporadic basal cell carcinoma. Vismodegib shows moderate efficacy rates in advanced BCC, 30% response rate in patients with metastatic basal cell carcinoma, and 43% in locally advanced basal cell carcinoma . We present 3 patients treated with vismodegib, characterizing the clinic evolution and discussing the role of vismodegib for each case. According to our experience, this drug represents a powerful tool for “advanced basal cell carcinoma”, with manageable side effects.

Venous lakes comprise vascular ectasias of the upper dermis, usually seen in the lips of older patients. Treatment can be sought for cosmetic or functional reasons and therapeutic options include laser ablation, cryotherapy or surgery. Electrosurgery is a frequent and efficacious method in the resolution of venous lakes, although possibly damaging to the adjacent skin. In order to minimize skin damage, an intravenous catheter can be used to deliver electrocoagulation intralesionally from an electroscalpel, being described as an improvement of the classic procedure. The authors describe this modified electrosurgical technique as a safe, simple and readily-accessible therapeutic option in the treatment of venous lakes.

A cutaneous collision or contiguous tumor occurs when two or more cutaneous tumors coexist in a single lesion. These tumors usually have misleading clinical and histological presentations, making them a diagnostic challenge. Although several different combinations of collisions have been described we report an unusual case of a cutaneous collision tumour on the back of the patient involving an intradermal naevus and a dermatofibroma. To the best of our knowledge, this is the first time such combination is reported.

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