Journal of the Portuguese Society of Dermatology and Venereology

The SPDV Journal is Changing

Margarida Gonçalo — 2021-12-24

Influence of Dysbiosis on Scalp Diseases

Francisca Morgado — 2021-12-27

Nos últimos anos vários estudos demonstraram a implicação da microbiota intestinal em várias doenças de mediação imune como a diabetes, a colite ulcerosa e a esclerose múltipla. Existem poucos dados sobre o microbioma folicular e o seu papel na patogénese de doenças que afetam o couro cabeludo, sendo uma área de investigação crescente. Alguns estudos mostram influência da disbiose nestas doenças, podendo a manipulação do microbioma representar uma possível opção terapêutica. Este artigo procura rever o conhecimento atual relativo ao impacto da disbiose nas doenças dermatológicas do couro cabeludo, como dermatite seborreica, psoríase, alopécia areata, alopécia androgenética, líquen plano pilar, alopécia fibrosante frontal e foliculite decalvante. Uma compreensão alargada deste tema poderá sugerir tratamentos adicionais além das terapêuticas convencionais.

Acute Vascular Reactive Inflammatory Acral Skin Lesions and SARS-CoV-2

Renan Minotto — 2021-12-24

Introduction: Coronavirus disease 2019 (COVID-19) predominantly affects the respiratory system, but there have been many reports of skin involvement. Several cutaneous manifestations associated with COVID-19 have been identified, including acral lesions (pernio-like), which we intend to better characterize in this study.

Methods: Demographic, clinical and major histopathologic characteristics of acral skin lesions occurring during the acute infection stage of COVID-19 disease were evaluated in this survey conducted in the Dermatology Division of Santa Casa Hospital Complex, Federal University of Health Sciences, Porto Alegre, Brazil.

Results: In the present study, we evaluated eight female patients with acute SARS-CoV-2 infection confirmed by PCR with acral skin lesions including pernio-like. The mean age was 43.6 and with no other acute disease, who had a good outcome with need for hospitalization only in 2 cases. Pernio-like lesions were observed under hot weather conditions in patients who denied any previous history of similar cutaneous manifestations. Some acral lesions resembled those of lupus erythematosus and of herpes simplex paronychia, but laboratory tests were negative for those conditions. Unusual nail involvement was present, characterized by half-moon shaped erythematous bands at the distal margin of the lunula.

Conclusion: Recognition of these skin lesions is important in that they could be potential markers, aiding in the diagnosis and prognosis of COVID-19 disease.

Vulvar Diseases that Required a Biopsy: A Retrospective Study

Rita Bouceiro-Mendes — 2021-12-27

Introduction: The vulvar area may be affected by many noninfectious conditions with similar clinical appearance, requiring a cutaneous biopsy. Our goal was to characterize the noninfectious vulvar diseases that required a biopsy in a southwestern Europe Central Hospital during a 10-year period.

Methods: A retrospective study of all the noninfectious vulvar diseases with histological confirmation diagnosed in our institution was conducted between January 1, 2008 and December 31, 2017.

Results: The sample included a total of 323 biopsies from 317 patients, aged between 11 and 98 years (mean age of 54.2 years). A total of 36 vulvar diseases was identified. Neoplastic conditions were the most frequently found, particularly melanotic macules (22.3%). The most frequent malignant tumor was vulvar intraepithelial neoplasia (6.2%) and squamous cell carcinoma (5.6%). The most common dermatosis was lichen sclerosus (12.7%).

Conclusion: Neoplasms were the most frequently diagnosed conditions affecting the vulvar area that required a biopsy. Ruling out malignancy was also the main reason to perform a biopsy. This study highlights the variety of noninfectious diseases that may affect the vulva and require a biopsy. Since vulvar diseases may be serious and carry high levels of patient distress a correct understanding of these conditions is crucial.

Determinants for Drug Survival of Methotrexate in Patients with Psoriasis: 10-Years Retrospective Analysis of DERMA.PT Registry

Nuno Gomes — 2021-12-27

Introduction: Methotrexate has been used in the systemic treatment of psoriasis since 1950 and remains a first-line drug. It is important to assess the factors that influence its discontinuation. The aim of the authors was to identify determinants of drug survival of metho- trexate in patients with psoriasis.

Methods: A retrospective analysis was performed concerning patients who started psoriasis treatment with methotrexate between January 2010 and January 2020 and were included in the national registry DERMA.PT by the Centro Hospitalar Universitário São João.

Results: A total of 146 patients with psoriasis treated with methotrexate alone or in combination with phototherapy were identified. Most were male (55%), with a mean age of 51±13 years and 65% had psoriasis for more than 10 years. Psoriasis vulgaris (49%) and psoriatic arthritis (47%) were the most common forms of psoriasis, with a mean initial PASI of 10.7±5.6. The majority of patients were methotrexate-naïve. In total, 66 (45%) patients discontinued treatment, with a mean survival time of 18.0±15.5 months. The most common reasons for discontinuation were ineffectiveness (32%), poor compliance (18%) and gastrointestinal intolerance (11%). Previous treatment with cyclosporine and the presence of psoriatic arthritis had both statistically significant associations with the discontinuation of methotrexate. In most cases, a switch to anti-TNFα monoclonal antibody was performed.

Discussion: In recent years, retrospective studies have been published focusing on the drug survival of methotrexate in psoriasis, ranging from 12 to 21 months. The main reasons for discontinuation were ineffectiveness and adverse effects, namely gastrointestinal. Our study is in line with what has been described but stands out for the high percentage of patients who maintained treatment. The tremendous evolution in the treatment of psoriasis over the 10-year period of the study greatly influenced the results. The main limitation of this analysis results from its re- trospective nature.

Conclusion: The results are in agreement with the published survival characteristics of methotrexate and reinforce its importance and role of this drug in the treatment of this disease.

Atopic Dermatitis: Epidemiological Profile and Associated Allergic Conditions - Data from a Referral Service in Southeastern Brazil

Carolina F. Pereira — 2021-12-27

Introduction: Atopic dermatitis is a chronic, inflammatory and pruritic dermatosis of high prevalence, especially in childhood, constituting a relevant problem for public health. Several factors are associated with an increased risk for atopic dermatitis: genetic, psychological, infectious, food, environmental, among others, and atopic dermatitis may be associated with other manifestations of atopy, such as asthma and rhinitis. This study aimed to determine the epidemiological profile and the prevalence of personal and family history of allergy in children with atopic dermatitis attended at a reference service, comparing it to the existing literature.

Methods: Hospital-based, cross-sectional and descriptive study, involving children of both genders under 18 years old with a clinical diagnosis of atopic dermatitis (Hanifin and Rajka criteria) attended at the Pediatric Dermatology Outpatient Clinic of the Hospital Municipal Universitário de Taubaté (H.MUT), SP – Brazil, from October 2018 to April 2019. Epidemiological data and data related to personal and family history of allergies were collected from medical records. Microsoft Excel 2019 was used for data analysis and compilation.

Results: Of the 440 consultations carried out in the period, 35 (7.9%) were for atopic dermatitis, 23 females (65.7%), 15 phototype IV (42.9%) with mean age 7.7 years (standard deviation=4.3), with disease duration from 2 months to 14 years (mean 5.3 years – standard devia- tion=4.3). Thirty-one patients (88.6%) manifested the condition up to the fifth year of life. Personal history of allergies was present in 77.1% (27 individuals), with a predominance of allergic rhinitis and in older age groups. Positive family history in 62.9% (22 individuals).

Conclusion: Pioneer study in the region; it allowed the determination of the epidemiological profile of patients with atopic dermatitis at the Pediatric Dermatology Clinic of the H.MUT, revealing an early onset and higher prevalence in females and in individuals with high phototypes. In addition, it identified the prevalence of personal and family history of allergy in these patients, data consistent with the literature.

Epidemiology and Evaluation of Diagnostic Methods in Superficial Mycoses in the Dermatology Service of a Public Hospital in Santos, Brazil

Fernanda J. Bauer — 2021-12-27

Introduction: Superficial mycoses are fungal infections caused mainly by dermatophytes, yeasts and non-dermatophyte filamentous fungi, which affect the most superficial layers of the skin and its appendages. They have a high prevalence worldwide.
The aim of this study is to evaluate the epidemiology of superficial mycoses, as well as the index of agreement between direct mycological exa- mination and fungal culture.

Methods: This is a retrospective study carried out at the Dermatology clinic of a tertiary hospital, during 6 years. For diagnostic elucidation, ma- terial was collected by scraping or curettage, for further analysis by direct mycological examination and culture for fungi.

Results: Four hundred thirty nine samples of suspicious lesions of superficial mycoses from 420 patients were included, 268 female (63.8% pa- tients) with a mean age of 45.7 years (3 months to 95 years), with most cases from the nails (43.4%) and glabrous skin (24.1%). In general, the most common fungus found in the culture was Trichophyton rubrum, however, not in all studied skin sites. Direct mycological examination showed a statistically significant association with culture (K=0.955), if cases with contamination on culture were eliminated.

Conclusion: Direct mycological examination and culture, as diagnostic methods in Dermatology, provide satisfactory and low-cost results, favo- ring patients and the health system. This study allowed us to describe the epidemiological profile of patients at a reference dermatology center, with relevant data in relation to our objective. Agreement between direct mycological examination and culture showed the reliability of the me- thods.

Sensitive Skin Syndrome: Literature Review of an Emerging Condition

Frederico Bonito — 2021-12-27

Sensitive skin syndrome (SSS) can be defined by the occurrence of unpleasant sensations such as stinging, burning, pain or itching after the skin is exposed to physical, chemical or thermal stimuli that would not normally cause these symptoms. With an estimated prevalence of up to 50% in women and up to 30% in men, this condition can be diagnosed using questionnaires and skin reactivity tests. The pathophysiology appears to involve a decrease in epidermal barrier function and neurosensory and immunological dysfunction. Treatment includes general measures, such as avoiding trigger factors, reducing the number of cosmetics applied, and using biocompatible moisturizers. Treatments targeting the signaling pathways affected in the disease, such as TRPV1 inhibitors, are under development.

Zoon Balanitis and Lichen Sclerosus: An Uncommon Association

Pedro Miguel Garrido — 2021-12-27

Zoon balanitis and lichen sclerosus are both chronic inflammatory disorders of the genital mucosa that usually affect middle-aged or elderly uncircumcised men.
Although the precise etiology of Zoon balanitis is still unclear, a pathogenic role of irritant and mechanical factors has been suggested. Therefore, foreskin sclerosis and phimosis caused by male genital lichen sclerosus may trigger the development of Zoon balanitis. However, until the present, only three cases with clinical and histopathologic features consistent with synchronous presentation of both disorders have been described.

We report the case of a 70-year-old male who developed Zoon balanitis in association with lichen sclerosus, that cleared only after circumcision.

A New Vision about Folliculitis Decalvans and Lichen Planopilaris: Two Distinct Entities or a Continuous Phenotypic Spectrum?

Ana Marcos-Pinto — 2021-12-27

Folliculitis decalvans and lichen planopilaris have been considered two distinct cicatricial alopecias. However, biphasic presentation of folliculitis decalvans - lichen planopilaris in the same patient has been recently described, therefore raising the doubt if they are two distinct entities or a continuous phenotypic spectrum.
We describe the case of a man who presented clinical, trichoscopic, and histopathological features of both entities. Moreover, we discuss current theories about the pathogenesis of the two diseases and their coexistence in the same patient. The recognition of similar cases allows to optimize the approach and treatment.

Trichilemmal Horn: A Case Report

Rodrigo C. Davanço — 2021-12-27

Trichilemmal horn or trichilemmal keratosis is a benign neoplasm, that occurs more frequently in females and in the age group over 50 years old. Clinically, it presents as a cutaneous horn and histologically shows trichilemmal keratinization at the base of the lesion.
As an uncommon pathology, in this case report, we will present the case of a young man (14 years old), presenting a keratotic lesion on the forehead for eight months, which revealed the diagnosis of trichilemmal horn after lesion exeresis and anatomopathological analysis.

Purpura Fulminans in a 20-Year-Old Female

Francisca Alves — 2021-12-27

A previously healthy 20-year-old female presented with extensive retiform purpura located at the face, upper and lower limbs, one week after an episode of acute tonsillitis. Despite the exuberance of the cutaneous findings and progression to skin necrosis she had no accompanying symptoms. Laboratory investigation revealed a heterozygous protein C mutation (exon 9, c.1332G> C, p.Trp444Cys), accounting for a partial deficiency of this anticoagulant protein. The patient was started on broad spectrum antibiotics, anticoagulation and systemic corticosteroids, with no lesional progression and complete resolution of cutaneous ulceration within 6 months.

This is a singular case of purpura fulminans, since two different causative factors precipitated the events. The previous tonsillitis reported by the patient is significant, because the serum concentration of protein S may also decrease after an infectious event - post-infectious purpura fulminans. This case illustrates that purpura fulminans due to autoantibodies against protein S, although rare, should be considered, especially in the absence of a severe acute infection. It also illustrates how in a given patient different independent factors can act simultaneously, triggering potentially devastating clinical scenarios.

Two Novel ATP2C1 Mutations in Portuguese Patients with Hailey-Hailey Disease

Sofia Antunes-Duarte — 2021-12-27

Hailey-Hailey disease (HHD) is a rare autosomal dominant acantholytic dermatosis. It is characterized by a recurrent eruption of vesicles, erosions, and scaly erythematous plaques involving intertriginous areas and first occurring after puberty, mostly in the third or fourth decade. In 2000, mutations in the ATP2C1 gene on band 3q22.1, encoding the secretory pathway Ca2+/Mn2+-ATPase protein 1(hSPCA1), have been identified as the cause of HHD. We report the identification of two novel mutations of ATP2C1 gene in two Portuguese patients, which expands the spectrum of ATP2C1 mutations underlying HHD and provides useful information for genetic counseling.

Topical Photodynamic Therapy as a Treatment Option for Periocular Bowen's Disease: A Case Report

Joel Reis — 2021-12-27

Cutaneous squamous cell carcinoma in situ of the eyelid and periorbital skin malignancies is common and its management is demanding. Surgical excision is considered the first-line treatment, but these techniques have limitations. Topical photodynamic therapy is currently approved for the treatment of squamous cell carcinoma in situ in other areas, but the reports of its use in this sensitive anatomic location area are scarce. Herein we report the case of a 61-year-old-man with extensive periocular Bowen disease that was treated successfully with photodynamic therapy. Photodynamic therapy may be an option as neoadjuvant or curative therapy in selected cases, especially in sensitive anatomic locations where surgery might lead to distortion of the structures or where techniques as micrographically controlled surgery are not available.

Cutaneous Primary Cryptococcosis in an Immunocompetent Patient: Case Report

Nicole S. Aranha — 2021-12-27

Cryptococcosis is a systemic infection caused by Cryptococcus neoformans, an encapsulated opportunistic yeast. It primarily causes significant infections in immunocompromised individuals and the symptoms vary according to the integrity of the immune system. Cutaneous cryptococcosis affects about 20% of patients with disseminated cryptococcosis, but primary cutaneous cryptococcosis (PCC) without systemic infection is rare.

A 76-year-old male patient with chronic obstructive pulmonary disease, hypertension and dyslipidemia, presented with a violaceus inflammatory skin plaque with blisters that progressed despite intravenous ceftriaxone for 7 days. Histopathology of an incisional biopsy was compatible with the diagnosis of cutaneous cryptococcosis. There was a complete response to fluconazole 300 mg/day for 3 months. No systemic disease was detected and there was no evidence of immunosuppression.

The importance of including cutaneous cryptococcosis in the differential diagnosis of skin lesions in patients without immunosuppression or the use of immunosuppressive therapy is emphasized. The cutaneous manifestations of the infection can be the first indication for a disseminated disease, therefore, its early recognition is essential to obtain a good prognosis.

Asymptomatic Nodules in a Child

Regina Caldas — 2021-12-27

Atypical Presentation of Herpes Simplex 1 Virus in a COVID-19 Patient

Maria José Guimarães — 2021-12-27

Idiopathic Pyoderma Gangrenosum Treated with Adalimumab: A Case of Success

Nuno Gomes — 2021-12-27