Repositório RCAAP

Introduction: Atopic dermatitis (AD) is a chronic inflammatory disease that affects both children and adults. The main symptom is pruritus, which has an important impact on quality of life (QoL). The objective of our study was to assess the impact of AD on the quality of life of children through the CDLQI score. Population and Methods: We performed a cross-sectional survey, with descriptive and analytical aims, carried out on the basis of prospective recruitment, carried out over a period of 7 months from December 2017 to June 2018. The study included children (5-16 years) with AD selected during dermatology consultations at the Centre Hospitalier Universitaire of Treichville. Data collected included the socio-demographic characteristics of patients and their AD and the results of a questionnaire on the severity score (SCORAD) and the quality of life index in these children (CDLQI). Results: We included 60 children, mainly within the group from 05 to 09 years (57%). CDLQI average score was 9.9 and mean SCORAD 24. Pruritus, insomnia and sadness had more impact on QoL. We found a strong association between the severity of AD objectified by SCORAD and the CDLQI. Conclusion: The study reveals a high impact of AD on the QoL of children in Abidjan mainly through pruritus, insomnia and sadness. It opens up reflection on the need for education for AD management and the prospect of creating an atopy school in Côte d’Ivoire.

Squamous cell carcinoma (SCC) is the second most common non-melanoma skin cancer and its incidence has been increasing over the past decades. SCC in-transit metastases are rare and predict a poor prognosis. We present the case of a 69-year-old patient with a right lower leg SCC, surgically excised with free margins. One month later, erythematous to violaceous, firm papules begin to erupt on the right lower leg, evolving to ulcerated nodules over a period of weeks. Homolateral inguinal and iliac nodal metastases were documented by percutaneous biopsy of an inguinal palpable lymph node and later documented by positron emission tomography – computed tomography (PET-CT). Given the unresectability of the disease, regional chemotherapy involving isolated limb perfusion (ILP) with melphalan and tumor necrosis factor-alpha (TNFα) was performed in order to avoid amputation. Macroscopic regression ensued, with complete resolution of all visible in-transit metastases 12 weeks after perfusion. However, a fatal outcome was observed 4 months later, due to systemic metastases. ILP is an effective and well-established technique in the treatment of advanced tumors of the extremities. Its main indications are in-transit metastases of malignant melanoma and advanced soft tissue sarcomas. However, studies have shown its effectiveness in other tumors such as locally advanced SCC. Despite the unfortunate outcome, it was possible to avoid amputation of the limb, with complete local disease remission.

Introduction: Between 2008 and 2019, in the Skin Pathology Laboratory of the Dermatology Department of Hospital de Santa Maria (Lisbon), 60 741 histological exams were performed, 11 873 of them concerned non tumour diseases (20%). Material and Methods: A retrospective study was conducted through the analysis of the histological exams of the non-tumoral skin pathology of the laboratory between January 2008 and December 2019. Results: Toxic erythemas were the most frequent group diagnosed (16%), followed by spongiotic dermatosis (14%), cutaneous infections (14%), psoriasiform dermatosis (10%) lichenoid dermatosis (8%), autoimmune bullous skin diseases (7%), vasculopathies (7%), collagenosis (6%), granulomatous diseases (5%), non tumour skin adnexal diseases (4%), panniculitis (1%), non tumour mucosal diseases (1%) and genodermatoses (1%). A number of cases (6%) corresponded to many diverse diagnoses that could not be classified into the main groups considered. Conclusion: Comparing with two previous series concerning the activity of this laboratory (referring to the period between 1975-1980 and 1998-2007), it was noticed that the total number of exams that were done in the considered period had consistently grown. This global growth was seen in all pathological categories, with the exception of genodermatoses. The observed results reaffirm the relevant role of the Skin Pathology Laboratory in the context of the Dermatology Department of Hospital de Santa Maria (Lisbon), not only based on the volume of exams there processed but also due to the higher level of expertise it achieved.

Milroy disease is a relatively rare condition of congenital primary chronic lymphedema that affects quality of life and requires medical follow-up due to the risk of secondary infections and malignancy. We report the case of a 49 years’ old female patient with an exuberant lymphedema in the lower limbs since her youth, progressing with unusual overlying skin lesions, as well as infectious complications. There are nine members of the family similarly affected, illustrating the pattern of autosomal dominant genetic inheritance of the disease.

Schwannoma is a benign encapsulated nerve sheath tumor composed of Schwann cells, that may arise anywhere along the course of a nerve. Cutaneous schwannomas, associated with peripheral nerves, usually present as a slow-growing well-circumscribed nodule, localized in the dermis or subcutaneous tissue. They are generally asymptomatic; however, they may become painful by the nervous compression. Since the clinical signs are non-specific, histology is required for a definitive diagnosis. Herein, we present a case of a solitary cutaneous ancient schwannoma of the ear, a histopathological variant of schwannoma with distinctive morphological characteristics.

Palmoplantar pustulosis (PPP) is a chronic and relapsing disease of the palms and soles, which tends to be difficult to treat. Classically it was described as a subtype of psoriasis. Nowadays it is regarded as a separate entity, although plaque-type psoriasis affects concomitantly many patients with PPP. A link between allergic contact dermatitis (ACD) and PPP has been described, and the former may encompass a potential to perpetuate the latter. Hereby we report two cases of difficult to treat plantar pustulosis. Underneath the dermatosis’s refractive nature was an allergic sensitization to potassium dichromate and cobalt chloride. Leather shoes and leather insoles were commonly worn, and both allergens are employed by the leather industry. Clinical remission was achieved in both cases with allergen eviction. Patch testing must be considered in cases of PPP without response to treatment. If relevant sensitization is found, allergen eviction measures are mandatory.

The reconstruction of nasal skin defects is a challenge even for an experienced dermatological surgeon. When the defect is too large or complex to be corrected with simpler flaps, interpolated flaps are a good option, but they usually require two surgeries. Tunneled interpolated flaps were developed to allow the reconstruction of complex defects in only one surgical procedure. In nasal reconstruction, the main tunneled flaps are the paramedian forehead and the melolabial flaps. This review will focus on these two flaps: description of the technique, applications, advantages and disadvantages.

Factitious disorder in dermatology is a dermatosis occuring with increasing frequency all over the world. Prevalence is reported around 2% in dermatology clinics but it is probably underdiagnosed, since the patient is not usually aware of its self-inflicted nature. When manifested by bullous lesions, it can be confused with autoimmune bullae. We report the case of a 59-year-old patient accompanied by psychiatry for severe depression with psychotic symptoms, dissociative amnesia, Munchausen syndrome and fibromyalgia who developed tense blisters on the dorsum of her left foot. The findings of the first histopathological examination suggested bullous pemphigoid and treatment with prednisone was performed. Due to the persistence and fixed location of the lesions, psychiatric history and negative direct immunofluorescence, a new histopathological examination was performed and interpreted as a case of bullous factitious disorder.

Introduction: Dupilumab is a fully human monoclonal antibody that blocks interleukin-4 and interleukin-13, key drivers of type 2 helper T-cell (Th2)-mediated inflammatory response. It was the first biologic treatment approved for adult patients with inadequately-controlled moderate-to-severe atopic dermatitis (AD). Continuous collection of daily data practice is important in order to evaluate the real effectiveness and safety of dupilumab treatment. Methods: In this observational cohort study, we prospectively included all adult patients with moderate to severe AD treated with dupilumab in our portuguese dermatology center from July 2019 to April 2020. Baseline clinical data was initially collected and treatment effectiveness and safety were assessed after 16 weeks. Results: Twenty-five patients were included. All patients had been previously treated with systemic immunosuppressants. The estimated mean Eczema Area and Severity Index Score (EASI) decreased from 27.8 at baseline to 8.8 at week 16 (+/- 4 weeks). A ΔEASI 75 response was achieved by 58.3% of patients (ΔEASI 90 - 29.1%). Conjunctivitis was the main reported side-effect, affecting 20.8% of patients. Discussion: Our study showed a significant EASI reduction during the first 16-weeks of dupilumab treatment in adult patients with AD. Despite its overall safety, daily-practice data tend to report a higher risk of conjunctivitis than previously expected and we hence recommend that patients should be specifically informed about this possible side-effect.

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Cholinergic urticaria is a relatively common condition defined by itching, redness and whealing induced by exercise and passive warming. In turn, acquired idiopathic generalized anhidrosis is a rare disorder of unknown pathogenesis, characterized by an impairment in total body sweating despite exposure to heat or exercise. We report two cases of this extremely rare association of cholinergic urticaria and acquired generalized hypohidrosis, and briefly review current knowledge with regard to classification, ethiopathogenesis and therapeutic options.

Novel coronavirus disease of 2019 (COVID-19) has impacted health care in general and has disrupted our routines. Health care services were mostly directed towards the emergency response and containment measures to halt virus spreading have been applied worldwide. It has also indirectly impacted the transmission of other infections, including sexually transmitted infections (STI). For STI, physical distancing measures have the potential to decrease STI transmission whereas the impact of COVID-19 on healthcare resources with interruption of STI diagnosis and treatment drives in the opposite direction. The question is how the counterbalancing impact of these forces will affect the course of STI epidemic. In this article we review the current available data on the COVID-19 impact on sexual behaviours, STI services interruption and reported STI rates, including local data.

Acute generalized exanthematous pustulosis is a rare drug-induced eruption that is characterized by sterile non-follicular pustules arising on an often edematous diffuse erythematous background. Generalized pustular psoriasis is an acute and severe clinical form of psoriasis presenting as pustular eruption with erythematous base. Differentiating both diseases is often challenging because of their clinical and histopathological similarities. A 62-year-old woman presented with pustular eruption and fever three days after taking propyphenazone. Laboratory findings revealed leukocytosis and neutrophilia. Histopathological findings revealed a subcorneal spongiform pustule filled with neutrophils and perivascular inflammatory cell infiltration with neutrophils in the dermis. She was initially treated with systemic steroids, however, the lesions showed insignificant improvement. Treatment was then shifted to methotrexate which resulted in a dramatic clinical improvement.

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Basal cell carcinoma is the most common skin cancer, with a very low incidence of metastases, associated with high morbidity and mortality. The morpheaform clinical variant is uncommon, presenting an aggressive growth pattern. Early diagnosis and new targeted therapies for metastatic disease are important to improve survival rates. We present the case of a 29-year-old patient with morpheaform basal cell carcinoma in the right genian region. Due to local recurrences, he underwent surgery and adjuvant radiotherapy. For extensive metastatic bone involvement, the patient started vismodegib and, later, pembrolizumab, and also performed palliative radiotherapy. Despite several lines of systemic therapy, the disease progressed, and the patient died after 8 years of follow-up. This case shows how crucial it is to identify risk factors for metastatic basal cell carcinoma and highlights the need to improve target therapies and tailor them to the patient’s biological profile.

Introduction: Vitiligo is an amelanotic disorder that can manifest as localized patches to near total body depigmentation. It is considered a cosmetic disease but it is accompanied by extensive psychological effects. Multiple treatments have been tried until now but no single method has achieved the desired response. Tacrolimus is frequently used for vitiligo treatment with few adverse effects. Microneedling is a newer modality of therapeutic wounding that augments drug absorption. Combination of microneedling and tacrolimus have been tried to improve the repigmentation response in vitiligo. Our objective was to assess the safety and efficacy of this combined modality. Methods: Forty patients were subjected to combination of tacrolimus 0.1% ointment and microneedling at 2 week intervals for maximum 10 sessions with a follow-up period of 6 months. Assessment was based on grading of repigmentation and clinical improvement according to vitiligo noticeability scale. Results: Excellent repigmentation was observed in 30% cases. Improvement was significantly higher in lesions present on the head and neck. Mucosal vitiligo also showed good to excellent response. No severe side effects were reported. No complications or recurrences were observed during the follow-up period. Conclusion: Combination of microneedling and tacrolimus was found to be safe and efficacious in the treatment of stable vitiligo.

Carcinoma cuniculatum is a rare variant of low-grade and well-differentiated squamous cell carcinoma. It is a locally invasive tumor, although it has low metastatic potential. It was originally described in the plantar region but may exceptionally appear in other locations. It predominates in middle-aged men and presents clinically as an exophytic, solitary, painful tumor, with insidious growth. Histologically, this tumor simulates a variety of benign dermatoses and may require several biopsies for the correct diagnosis. It is often misdiagnosed as a viral wart, due to the clinical similarity and the indolent course. The treatment of choice is surgical excision due to the high risk of recurrence and locally aggressive behavior. Amputation can be performed in cases of deep tissue invasion. We present a case of carcinoma cuniculatum in which the late diagnosis favored the invasion of the underlying bone, resulting in amputation of the affected finger.

Metastatic melanoma has been associated with a poor prognosis, with overall survival rates at 5 years of 10%. Until 2011, the only treatments available for metastatic melanoma were chemotherapy and immunotherapy with interleukin-2. The more in-depth knowledge about the molecular biology of melanoma and the identification of BRAF mutations, which are the most frequently found, allowed us to find new therapeutic targets that came to modify the prognosis of these patients. Currently, the treatments available for metastatic melanoma with BRAF mutation are immunotherapy with immunological checkpoint inhibitors (anti-PD-1 to anti-CTLA-4) and targeted therapy with BRAF inhibitors and MEK inhibitors. However, the first-line therapy to be instituted in these patients remains unknown.

Introduction: Keloids are benign skin tumors caused by changes in the wound healing process. There are various therapeutic options available and the combination of techniques is commonly used. Conclusion: Five patients underwent a combined treatment of surgical resection of a keloid in the earlobe with subsequent placement of a pressure earring and were evaluated using sterile plungers of 5 mL syringes. The procedures were performed at Jean Bitar Hospital in the state of Pará from 2019 to 2020. No local recurrence was observed. The earring production was considered low cost, easily reproducible, good aesthetic appearance, and good tolerability.