Repositório RCAAP

Background: Eyelid dermatitis is a common disease and its etiology frequently difficult to ascertain, although allergic contact dermatitis is the most common cause in patch-tested patients. This is a 5-year retrospective study to evaluate the most frequent causes and allergens in patients with allergic eyelid dermatitis.Materials and Methods: Using a computerized database, patients with eyelid dermatitis patch tested over a 5-year period from 2009-2013 on the Dermatology Consultation of Hospital Curry Cabral were identified.Results: Over the 5-year period (2009-2013) from a total of 1341 patch tested patients, 117 (8.7%) had eyelid dermatitis. The most frequent etiology was allergic eyelid dermatitis (54 patients), followed by atopic dermatitis (22 patients). Toluenesulfonamide Formaldehyde resin was the most frequent allergen identified. The preservative allergens group was the most frequently identified, followed by fragrances and drugs.

Junctional epidermolysis bullosa is a group of inherited blistering diseases characterized by increased skin fragility, blisters and erosions after minor trauma, due to tissue cleavage at the dermal-epidermal junction. We report the case of a male Caucasian infant, born with erosions and tense blisters on the hands, abdomen and scalp. Immunoflurescence antigen mapping revealed paucity of collagen XVII immunolabelling, compatible with the diagnosis of non-Herlitz junctional epidermolysis bullosa. Molecular analysis of the COL17A1 gene revealed compound heterozygosity for two frameshift mutations. Family history was positive for a brother with a bullous dermatosis, who died from sepsis on the 3rd week of life without a definitive diagnosis that could enable precise risk estimation and prenatal diagnosis. We emphasize the importance of rapid referral to specialized centers, to provide early accurate diagnosis, adequate clinical management and prenatal diagnosis in subsequent pregnancies.

Uremic calciphylaxis is a rare but serious complication of dialysis patients. Its pathogenesis is not completely understood, although many risk factors have been identified, such as hyperparathyroidism. Cinacalcet (a calcimimetic agent) is used to keep calcium, phosphorus and PTH within the normal range. A 72-year old female patient, under hemodialysis for 15 years with hyperphosphatemia, normocalcemia and hyperparathyroidism despite therapy with cinacalcet and sevelamer, presented with several painful ulcerated lesions of the right leg, with a geographical configuration, central necrotic surface and hemorrhagic borders that progressed for 6 weeks. Cutaneous biopsy revealed vascular deposits of calcium in deep dermal and hypodermal vessels, confirming the clinical diagnosis of calciphylaxis. The necrotic borders of the ulcer improved when cinacalcet and sevelamer were administered in maximum doses. However, the patient passed away in consequence of comorbidities. We report a case in which cinacalcet was ineffective in controlling the phosphate and calcium abnormalities in an hemodialysis patient and, consequently, his cutaneous lesions.

A 35-year old black woman was referred to a Dermatology appointment due to subcutaneous nodules located in the right paraumbilical region and right flank that had appeared 2 weeks before. Her medical history was relevant for renal insuficiency on hemodialysis and infection by the human immunodeficiency virus (HIV-1). On physical examination there were two well-defined subcutaneous nodules; upon palpation, these lesions had a hard consistency, were painful and not adherent to the deep planes. An incisional biopsy was performed and histopathological examination was consistent with the diagnostic hypothesis of calcinosis cutis. A careful review of all medication established a temporal relationship between the skin nodules and subcutaneous administration of nadroparin calcium in that location. The nodules resolved spontaneously within 2 months after stopping the nadroparin calcium subcutaneous injections. Calcinosis cutis due to subcutaneous administration of low-molecular-weight calcium-containing heparins is rare and it is assumed that an elevated calcium-phosphorus product may be decisive in its pathophysiology. It is generally self-limited, resolving spontaneously.

The purpura and cyanosis are elementary lesions frequently seen by medical doctors of several specialties. Its causes can range from rare diseases, such as thrombophilia, to the occupational injuries. The fundamentals of the formation of these lesions should be domain of dermatologists so that they can aid in the correct diagnosis of the underlying disease. The authors report the case of a patient in hospital who had cyanosis and ecchymosis in the digits caused by repetitive trauma to fingers in the measurement of capillary blood glucose. The differential diagnosis of the case is reviewed.

The association of linear IgA bullous dermatosis (LABD) with ulcerative colitis (UC) is defined, although the etiology of this association is still unclear. We report the case of a 41 year-old female patient with UC diagnosed 6 years ago with a crisis 2 months ago, referred for pruriginous and tense bullae with 1 month of evolution. Her past medical history was relevant for gestational diabetes, reactive depression and hypertension. She was medicated with mesalazine for 1,5 years, bisoprolol, mexazolam and drospirenone/ethinyl estradiol. Cutaneous biopsy showed a bulla on the dermoepidermal junction (DEJ) and a polymorphous inflammatory dermal infiltrate. Linear immunoreactivity for IgA on the DEJ confirmed the diagnosis of LABD. Prednisolone and dapsone were initiated with good clinical response, maintained after six months of follow-up. The aetiology of this association remains unknown, being hypothesised cross-reactivity of IgA idiotype from UC with the dermoepidermal antigens. Mesalazine imputability cannot be surely ruledout as the cause of LABD in this patient.

During the last decades, the incidence of opportunistic fungal infections has been increasing, namely in the context of immunosuppression. The Scedosporium apiospermum is a ubiquitous filamentous fungus in soil, decaying vegetation, sewers and polluted waters. Can cause infections in immunocompetent patients after trauma and severe and potentially fatal infections in immunocompromised patients. The authors describe a case of an infection by S. apiospermum on the dorsum of the left foot of a patient under prolonged systemic corticosteroid therapy combined with methotrexate due to the diagnosis of giant cell arteritis. Treatment of these infections can be a challenge due to the inherent resistance to many of the available systemic antifungal agents, including amphotericin B. In the case described, therapy with voriconazole was performed, with complete resolution of the lesions.

Onychocola canadensis is a non-dermatophyte fungus that has been reported to cause onychomycosis in elderly patients. Cutaneous infection is extremely rare. Because of its slow growth and rarity, its identification is difficult, potentially giving rise to falsely negative mycologic cultures. A 65-year-old male patient presents with an exsudative circular erythematous lesion, with 3cm, on the lateral border of his right hand. The lesion was evolving for one week, with later appearance of 2 similar lesions on the 4th finger and ipsilateral forearm. A biopsy was performed and material was sent to culture. The mycologic examination revealed the growth of Onychocola canadensis. The patient was treated with oral itraconazol for 2 months, with complete resolution.

Introduction: Follicular lymphoma is the most common B-cell lymphoma to occur as a primary tumor of skin.Case report: The case of a 69-year-old caucasian female patient, with a 1 year history of a painless nodule, localized in the frontal region, which had been observed several times on urgency room. On examination, we observed a nontender nodule, 3x2 cm, localized in the frontal region with small papules at the periphery. Histopathologic examination of a biopsy from the nodule was suggestive of follicular lymphoma.Immunophenotyping: CD20+, CD3-, CD5-, CD10+, Bcl2+ in rare cells. The patient was observed by Hematology/ Oncology and the staging was negative and a course of palliative chemotherapy with R-CHOP, followed by radiotherapy was given. During a follow-up of 6 months there wasn´t any evidence of recurrences.Conclusion: This case report intends emphasize the relevance of educate the doctors for the diagnosis of this disease.

Breast angiosarcoma is a rare tumor that can be divided in 3 groups: primary, secondary to chronic lymphoedema and secondary to radiotherapy (RT). It has a poor prognosis and a bad response to surgery and chemotherapy (QT). A female patient was observed with a very indurated erythematous-violaceous plaque, with an ecchymotic halo that occupied the right breast, and was evolving for 8 months. The patient had history of breast cancer, treated with surgery (tumourectomy) and local RT 5 years before. The incisional biopsy showed a vascular proliferation, arranged in nodules but also with diffuse growing, with cytological atypia and strong expression of CD31, CD34 and factor VIII, consistent with post-RT angiosarcoma. The patient begun RT and QT, but had no significant clinical response. She is now waiting for approval to begin pazopanib.

Ductal eccrine carcinoma is a rare and aggressive skin cancer. Its diagnosis implies the exclusion of a primary adenocarcinoma located elsewhere with cutaneous metastization, as the histological and immunohistochemical analysis are not sufficient by themselves. We report the clinical case of a 75-year-old male with a 3 cm width tumor located on the right hemiface, which was excised and whose histology and immunohistochemistry pointed towards an eccrine ductal carcinoma. The complementary investigation showed multiple pleural and pulmonary nodules, as well as neoplastic involvement of the dorsal and lumbar vertebral column. Their histological and immnunohistochemical examination, supplement with the absence of imagiologic findings suggesting a primary tumor in another location, allowed the diagnosis of a metastatic eccrine ductal carcinoma. The patient died 6 months after the initial consultation.

The increasing number of clinical trials in dermatology requires from the dermatologist the ability to evaluate the quality, understand, and synthesize information from multiple sources in order to make decisions regarding the therapeutic/diagnostic strategies. This medical education article explains basic principles, relevant and adapted to the dermatology area, fundamental to an appropriate interpretation of outcomes of clinical trials. Part I of this article looks at the different study designs and their impact on results.

Presently, medical practice - increasingly scrutinized by society - is marked by several constraints and limitations of financial, regulatory, bureaucratic and administrative nature. This restrictive, highly regulated environment, that does subvert classical medical practice, vividly contrasts with the laxity by which diverse nonqualified paramedic professionals of the aesthetic area and of the so called complementary and alternative forms of medicine are allowed to practice. The growing area of Cosmetic Dermatology is diversely and not without controversy judged by dermatologists, as a result of the multiple raised unanswered questions that pertain to patient-doctor relationship, litigancy, ethics and deontology. In an open, reflexive fashion, the main challenges ahead that mainly concern regulatory, judicial and ethical issues are presented. Lastly, some practical recommendations are suggested.

Chronic urticaria is a skin disorder with a very high impact on patients’ quality of life. It has a prevalence between 7,8 and 22,3% with a higher incidence in women between 20 and 40 years old. The intensity of the itch, the anxiety caused by its recurrent nature and also the incapacity caused by the unpredictable exacerbations are among the factors that mostly impact patient’s quality of life. Being a mast cell-driven disease, the most recent recommendations of the European Academy of Allergy and Clinical Immunology (EAACI), the EU-funded network of excellence, the Global Allergy and Asthma European Network (GA2LEN), the European Dermatology Forum (EDF), and the World Allergy Organization (WAO) suggest a symptomatic treatment for chronic urticaria, with very limited routine diagnostic measures being recommended. Non-sedating anti-histamines H1 (AHH1ns), in approved dose, are recommended as first line of treatment followed by the utilization of omalizumab, cyclosporine A or montelukast as add-on treatment in patients that do not respond to high doses AHH1ns. The treatment must continue until the patient is symptoms free, in the most possible safe manner.

Ultrasonography gained a wider applicability in Dermatology with the emergence of higher frequency probes that allow the visualization of lesions in the skin and subcutaneous tissue. High-resolution ultrasound has developed particularly in the field of malignant melanoma, and has positioned itself not only as an image complement to physical examination, but also as a locoregional staging technique. In fact, ultrasonography is more sensitive than lymph node palpation, it is superior to other imaging techniques when it comes to the early detection of locoregional metastasis and, when performed preliminarily in combination with fine needle aspiration cytology, it may reduce the number of sentinel- -node biopsies. Presently, available studies suggest that ultrasound has a relevant part in the evaluation of locoregional disease, particularly in patients with intermediate or high-risk cutaneous melanoma.

Introduction: Atypical fibroxanthoma (AFX) is a rare cutaneous tumor without a completely defined histogenesis which frequently develops on head and neck of elderly people. Its diagnosis is histological, of exclusion with other tumors with identical morphology. Despite its classical benign behavior some rare cases reporting distant metastasis classified it on intermediate malignancy.Material and methods: We preformed a retrospective analysis with purpose of reviewing the AFX diagnosis made in our department between 2008 and 2014. We analyzed demographic, clinical, histological and immunohistochemical data and also surgical treatment and recurrence.Results: We obtained 12 cases of AFX, 11 males and 1 female, the youngest was 25 years old and the others had a median age of 76 years. The lesions were nodules or tumors with 1.9cm median size, all of them located on the head. Histology revealed well defined lesions with atypical pleomorphic cells and frequent solar elastosis, classified as mix variant (spindle and histiocytic cells) in all cases. Immunohistochemical study showed positivity for vimentin and negativity for cytokeratins and S100 protein in all cases and CD10 and CD99 were frequently positive. Surgical excision was performed in 10 cases with 10% rate of recurrence during 3 years of median follow-up.Conclusions: These results were similar with other published series. This tumor is clinically unspecified and histologically undifferentiated and so immunohistochemistry is essential for diagnosis, however, for now, available makers are not sufficiently specific. Despite its good prognosis in most cases clinical surveillance is recommended.

Introduction: Fibrous histiocytoma represents a group of common benign lesions that occur mainly on the extremities. The location on the face is rare and here, more aggressive variants and higher local recurrence are described.Methods: We conducted a retrospective clinicopathological study of cases of fibrous histiocytoma arising on the face at the Dermatology Service of the Hospital Egas Moniz and the “Centro de Dermatologia Médico-Cirúrgica” in Lisbon, between January 2001 and December 2012.Results: In a total of 1307 fibrous histiocytoma (any location), 12 were located on the face. These lesions occurred in 8 females and 4 males (mean age 48.9 years). The preferred location was the nose (4 cases) followed by the chin (3 cases), interciliar (3 cases), infraorbital (1 case) and ear (1case). The most common clinical diagnosis was epidermoid cyst (5 cases). Histologically, the majority of tumors (10 cases) reached the deep dermis. Six cases showed common morphology. Three cases corresponded to the cellular variant of fibrous histiocytoma, two cases aneurysmal fibrous histiocytoma and one case hemosiderotic fibrous histiocytoma. In five cases mild pleomorphism and mitotic activity were evident. Necrosis, neural and / or vascular invasion were not identified. In seven lesions immunohistochemical studies for differential diagnosis were performed.Conclusion: In most cases correlation with the clinical diagnosis was not observed. Compared with common fibrous histiocytoma, involvement of deep structures, cell density and pleomorphism are more frequent, leading to a need of greater clinical vigilance and excision with wider safety margins.

Introduction: The incidence of malignant melanoma has been rising. Therefore, the knowledge of its prevalence is very important. The authors intended to study the prevalence, demographic variables and the clinical features of the malignant melanoma diagnosed in their department.Material and methods: A retrospective study of the MM diagnosed between 2006 and 2013 was performed. The data was statistically an analysed.Results: three hundred and forty one malignant melanomas were diagnosed in 302 patients, 189 in women. The average annual growth rate was 8.57%, with 27 MM in 2006 and 48 in 2013. The older age groups predominated. Overall the trunk was the most frequent location. The Breslow thickness averaged 1.18mm. A statistically significant increase in the proportion of thin malignant melanomas (<1mm) was found. Nevertheless, the malignant melanomas >2mm remained unaltered. Nodal disease (stage III) was evident in 36 patients. Only two patients had metastatic disease (stage IV).Discussion: The number of malignant melanomas diagnosed grew above the reported national and international incidence growth. The average Breslow thickness, above 1 mm, remains high. Nonetheless, the proportional increase in malignant melanomas <1mm points towards a positive evolution.Conclusion: Our data, though representing a limited population, may lead to conclusions on the efficacy of the prevention strategies regarding malignant melanoma.

Anogenital warts (AGW) are benign tumors of the skin and mucosal epithelium caused by human papilloma virus infection. There is a growing prevalence of anogenital warts in the pediatric population, resulting from vertical transmission, auto or heteroinoculation. The diagnosis is primarily clinical and biopsy is reserved for doubtful diagnosis. Most lesions resolve spontaneously over time but recurrence is frequent, even after treatment, so watchful waiting is a valid option. The possibility of sexual abuse in children with anogenital warts represents a challenge in clinical practice. Most authors consider that the diagnosis of sexual abuse relies mainly on a complete anamnesis, a skilled interviewer and a thorough physical examination. There is general consensus that the probability of abuse increases with the child’s age, especially over 5 years of age. For children under 2 years, nonsexual human papilloma virus transmission should be strongly considered, in the absence of traumatic signs, other sexually transmitted diseases or a consistent history of abuse. However, sexual abuse cannot be ruled out at any age, so a careful follow-up is required, considering all the previously mentioned factors.

Juvenile scleroderma is a disease that has different features in comparison to scleroderma in adults. Its incidence is rare on infancy. It can be systemic or localized and, this last form is more frequent. We report a case of juvenile scleroderma in a 3-year-old female child presenting with a yellow plaque on the trunk simulating a xantomathous lesion. The lilac ring around the lesion contributed to the construction of the correct diagnostic hypothesis which was confirmed by the histopathology. The precocious diagnosis on this case, allowed the beginning of the treatment on an initial phase of the disease, contributing to a better prognosis for the patient.