[Translated article] Iberia Consensus on Strategies to Prevent and Manage Irritation by Topical Retinoids in Facial and Trunk Acne.

OBJECTIVE: To assess the level of agreement on various prevention and management strategies for irritation caused by topical retinoids in facial and trunk acne in an attempt to alleviate it and minimize treatment discontinuations as much as possible. METHOD: After reviewing the scientific medical literatura currently available, 4 different areas of uncertainty in the management of irritation caused by topical retinoids in acne were identified. A questionnaire with 34 recommendations was created and evaluated by a group of 133 dermatologists (Delphi methodology). RESULTS: In 82.3% of the recommendations (28 out of 34), some level of agreement was reached (≥85% agreement in 22 recommendations and≥70% agreement in 6). The results with the highest level of agreement focused on specific patient education strategies (explaining that irritation is an expected reaction at the beginning of treatment and tends to decrease over time), gradual and/or spaced application of topical retinoids (at night time to prevent and/or reduce skin irritation), and the importance of using adjuvant products, specific for acne-prone skin, hydration, photoprotection, and skin cleansing. These recommendations reflect a comprehensive approach to managing irritation associated with topical retinoids and promoting long-term adherence. CONCLUSIONS: Skin irritation caused by topical retinoids in facial and trunk acne is an expected, mild, and controllable reaction if proper prevention and management guidelines are followed, meaning that it should not be a reason for treatment discontinuation.

Mechanism of acitretin-induced relaxations in isolated rat thoracic aorta preparations.

Acitretin is a member of vitamin A-derived retinoids, and its effect on vascular smooth muscle had not yet been studied. The aim of this study is to investigate the effect of acitretin, a retinoid, on vascular smooth muscle contractility. Thoracic aorta preparations obtained from 34 male Sprague-Dawley rats (355 ± 15 g) were studied in isolated organ baths containing Krebs-Henseleit solution. The relaxation responses were obtained with acitretin (10-12-10-4 M) in endothelium-preserved and endothelium-denuded aorta preparations precontracted with submaximal concentration of phenylephrine (10-6 M). The role of retinoic acid receptors (RARs), nitric oxide, adenylyl, and guanylyl cyclase enzymes, and potassium channels in these relaxation responses were investigated. Acitretin produced concentration-dependent relaxations, which were independent of its solvent dimethylsulfoxide (DMSO), in endothelium-denuded phenylephrine-precontracted thoracic aorta preparations. While incubation with the RAR antagonist (AGN193109, 10-5 M) had no effect on these relaxations; nitric oxide synthase inhibitor (L-NG-Nitro arginine methyl ester (L-NAME), 10-4 M), adenylyl cyclase inhibitor (SQ2253, 10-5 M), guanylyl cyclase inhibitor (oxadiazolo [4,3-a] quinoxalin-1-one (ODQ), 10-6 M), and potassium channel blocker (tetraethylammonium (TEA), 10-2 M) significantly eliminated the relaxation responses induced by acitretin. Acitretin induces relaxation in rat isolated thoracic aorta preparations without endothelium, which may be mediated by nitric oxide, cyclic adenosine monophosphate, and cyclic guanosine monophosphate-dependent kinases and potassium channels.

[Acute pancreatitis under acitretin]. / Pancréatite aiguë sous acitrétine.

INTRODUCTION: Retinoids are vitamin A derivatives with numerous indications in dermatology. Acute pancreatitis is a rare adverse effect of systemic retinoids. We report a case occurring during acitretin treatment for psoriasis. PATIENTS AND METHODS: A 27-year-old male patient with no history of diabetes, obesity, alcohol consumption or medication consulted for extensive pustular psoriasis. The lipid balance and liver tests were normal. The patient was treated with acitretin at a dose of 25mg/d. Four days after the start of treatment, the patient was admitted to the surgical emergency room for piercing epigastric pain with vomiting of bile, without transit problems. Serum lipase was 20 times the normal value (1278 IU/L). CRP was raised at 155mg/L and triglycerides were normal at 0.66g/L. Ranson's score was 1 and the abdominal scan revealed Balthazar Grade B pancreatitis with a small amount of peritoneal effusion. The ultrasound examination showed absence of gall stones, without dilation of either the intra- or extra-hepatic bile ducts. Acitretin was discontinued due to its possible causative role. The patient was treated by means of parenteral feeding, strict fasting and a proton-pump inhibitor, and a good clinical outcome with gradual normalization of serum lipase and CRP was achieved in 10 days. The patient was subsequently treated with infliximab for psoriasis, with good results. DISCUSSION: In the event of acute abdominal pain in a patient treated with retinoids, a diagnosis of acute pancreatitis should be considered. This complication can occur in the absence of hypertriglyceridemia.

[Acitretin-induced capillary leak syndrome: Case report and literature review]. / Syndrome de fuite capillaire observé sous acitrétine : un cas et revue de la littérature.

BACKGROUND: Retinoids are widely used in dermatology. Adverse effects are frequent and require clinical and laboratory monitoring. Herein we report the case of a patient with secondary capillary leak syndrome (SCLS) associated with acitretin. We then present a review of the literature on systemic retinoids and SFCS. PATIENTS AND METHODS: A 57-year-old patient consulted following the onset of severe type I pityriasis rubra pilaris. Treatment was initiated comprising topical corticosteroids combined with acitretin at a dose of 0.5mg/kg/day. On the eighth day, voluminous edema appeared, accompanied by weight gain of 8kg in 48h and hypotension. The laboratory assessment showed hypoalbuminemia and hemoconcentration. Acitretin-induced SCLS was diagnosed based on the triple signs of hemoconcentration, hypoalbuminemia and hypotension, as well as rapid improvement following discontinuation of acitretin. DISCUSSION: We collected 7 published clinical cases between 1981 and 2018, including our own case report. Retinoids were indicated only in severe cutaneous diseases. The mean time to onset of SLCS is 9.8 days, with a return to normal 17 days after discontinuation of retinoids. Capillary leak syndrome is a rare and under-diagnosed clinical-laboratory syndrome that must be recognized in order to avoid potentially fatal inappropriate management. It is a rare adverse effect of retinoids used in dermatology and the pathophysiology remains unclear.

[Pityriasis rubra pilaris]. / Pityriasis rubra pilaire.

Pityriasis rubra pilaris is a rare heterogeneous dermatosis associating three clinical signs to different degrees: follicular corneal papules, reddish-orange palmoplantar keratoderma and erythematosquamous lesions that may in some cases be very extensive, interspersed with patches of healthy skin. The aetiology is unclear, and in most cases, the trigger factors consist of trauma or infection, probably in subjects with an existing predisposition. In other cases, the condition is associated with immunological disorders or, in familial cases, genetic keratinisation abnormalities similar to ichthyosis. Given the widely varying signs, several classifications have been proposed, based on clinical criteria and outcomes. The outcome varies in accordance with the clinical forms involved. Therapeutic approaches are poorly qualified and there have been no clinical trials due to the rarity of the disease. However, the best results appear to have been obtained using oral retinoids, with second-line therapy comprising methotrexate and cyclosporine. The landscape of therapeutic strategy seems to be changing with the advent of new anti-tumour necrosis factor and anti-interleukin-12/23 antibodies.

Acral Hemorrhagic Darier Disease. / Enfermedad de Darier acral hemorrágica.

Darier disease is an autosomal-dominant inherited condition caused by mutation of a gene, which produces a protein involved in calcium channel regulation. The disease has a variety of manifestations and lacks consistent genotype-phenotype correlations. Acral hemorrhagic Darier disease causes macules, papules, vesicles and/or hemorrhagic blisters on the extremities. Other classic signs of the disease may be present in the same patient or relatives. Histopathology reveals dyskeratosis and suprabasal acantholysis with hemorrhagic lacunae. We report 3 new cases of this type of Darier disease triggered by injuries. Response to retinoid therapy was good.

Consensus-Based Acne Classification System and Treatment Algorithm for Spain. / Consenso español para establecer una clasificación y un algoritmo de tratamiento del acné.

Acne is a chronic inflammatory disease whose psychosocial effects can greatly impair quality of life. Various scales are used to classify the severity of acne, and several treatment algorithms are currently applied: no consensus on a common scale or treatment guidelines has been reached. A group of Spanish experts therefore met to identify a scale the majority could accept as the most appropriate for classifying severity and treating accordingly. The group chose the following classifications: comedonal acne, mild or moderate papulopustular acne, severe papulopustular acne, moderate nodular acne, and nodular-cystic acne (or acne tending to leave scars). Consensus was reached on first- and second-choice treatments for each type and on maintenance treatment. The experts also issued specific recommendations on antibiotic use (starting with mild or moderate papulopustular acne), always in combination with retinoids and/or benzoyl peroxide. The use of isotretinoin (starting at severe papulopustular or moderate nodular acne) was also covered.

Assessment of the efficacy and safety of a combination of 2 topical retinoids (RetinSphere) in maintaining post-treatment response of acne to oral isotretinoin.

INTRODUCTION: The high rate of relapse of acne lesions following oral isotretinoin treatment is a common problem which remains unsolved. To avoid or minimize relapses, topical retinoids have been used for many years as maintenance treatment. However, adverse effects frequently occur. AIMS: To determine the efficacy and safety of a new retinoid combination (Retinsphere technology) in maintaining post-treatment response to oral isotretinoin. PATIENTS AND METHODS: Prospective, randomized, double-blind and vehicle-controlled study of 30 patients with acne previously treated with isotretinoin. Treatment with the retinoid combination was applied to one side of the face and vehicle was applied to the other, once daily, for 3 months. Standardized photographs were taken using RBX technology at baseline, 1.5 months and 3 months. The primary efficacy endpoint was the appearance of relapse on the treated side compared to the vehicle-treated side. Other endpoints included lesion count, investigator-reported improvement, patient-reported improvement, impact on quality-of-life, and side effects. RESULTS: Although the majority of patients did not reach the total target dose of oral isotretinoin, the relapse rate was significantly lower on the retinoid-treated side compared to the vehicle-treated side. Likewise, improved lesion count and excellent tolerance were observed. CONCLUSIONS: This new retinoid combination (Retinsphere technology) were effective and safe as maintenance therapy after post-treatment response to oral isotretinoin in patients with acne.

Treatment of oral lichen planus. Systematic review and therapeutic guide. / Tratamiento del liquen plano oral. Revisión sistemática y protocolo de actuación.

In this systematic review, 55 structured articles on the therapeutic efficacy against pain and clinical signs of oral lichen planus (OLP) were analysed. The literature search was developed according to the criteria of the PRISMA system, selecting the tests performed using one of the following methodological designs: drug (active ingredient) vs. drug in different excipient or concentration, drug vs. different active principle, drug vs. phytotherapy and drug vs. treatment with phototherapy. Based on the results, an algorithm is proposed to guide the treatment of OLP in its atrophic and erosive clinical forms. The use of clobetasol propionate at 0.025-0.05% of topical application as the first therapeutic alternative is highlighted. Secondly, 0.1% tacrolimus and 1% pimecrolimus also formulated for its topical regimen. And finally, we address the use of systemic corticosteroids and the application of diode lasers.

Pitiriasis rubra pilaris atípica en pediatría. A propósito de un caso / Atypical pityriasis rubra pilaris in pediatrics. A case report

Resumen Pitiriasis rubra pilaris es una dermatosis eritematoescamosa infrecuente, de etiología desconocida producida por una alteración en la queratinización de la epidermis. Presenta una distribución bimodal con mayor incidencia en la primera y sexta década de vida. Posee una clínica heterogénea clasificada en 6 subtipos según Griffiths, de acuerdo a su presentación clínica y pronóstico. Sus principales hallazgos son pápulas hiperqueratósicas foliculares, queratodermia palmoplantar y placas eritematoesmamosas rojo-anaranjadas que pueden progresar a eritrodermia, con islas de piel sana. En niños las manifestaciones clínicas más frecuentes son la III y IV de la clasificación de Griffiths, según distintos estudios. La histología no es específica pero apoya el diagnóstico. Existen múltiples opciones terapéuticas según la extensión y severidad del cuadro. Presentamos el caso de un preescolar de 5 años de edad con diagnóstico de PRP atípica asociado a eritema extenso, con buena respuesta a corticoides sistémicos y posteriormente a retinoides tópicos.

Summary Pityriasis rubra pilaris (PRP) is an unusual erythematous squamous dermatosis of unknown etiology, caused by an alteration of keratinization in the epidermis. This disease presents a bimodal distribution, being its incidence greater in the first and sixth decade of life. It has a heterogeneous clinical manifestation, and, according to Griffiths, has been classified into 6 subtypes, based on clinical features and prognosis. The typical manifestations of this disease are follicular hyperkeratotic papules, palmoplantar keratoderma and orange-red scaly plaques that can progress to erythroderma, with islands of sparing. According to different studies, the most frequent clinical manifestations in children are type III and IV according to Griffiths classification. Histology is not specific but supports diagnosis. There are multiple therapeutic options, depending on the extension and severity of the disorder. This review presents the case of a 5-year- old child case with a diagnosis of atypical PRP associated with extensive erythema, his response to treatment of systemic corticosteroids and later to topical retinoids being good.

Retinoids for prevention and treatment of actinic keratosis / Retinoides para a prevencao e tratamento das queratoses actinicas

Actinic keratosis is a common cause of dermatological consultations and it presents a strong association with squamous cell carcinoma. Many substances are used for treatment and prevention, such as retinoids. Nevertheless, many studies on retinoids emphasize their application in treating and preventing non melanoma skin cancers. In this article, we reviewed studies about systemic and topical retinoids used with immunocompetent patients and organ transplant recipients with actinic keratosis, as primary or secondary outcomes. The majority of these papers pointed to a reduction in actinic keratosis count after treatment with retinoids. However, studies need to be better-defined in order to address the lack of a standardized dose, the absence of control groups, the low number of patients and short follow-up periods. Blind, randomized and controlled clinical trials with adequate sample sizes, specifically focused on actinic keratosis, are needed to clarify the real benefit of topical and/or oral retinoids. Comparison of efficacy and safety between oral and topical retinoids in the prevention and treatment of non-melanoma skin cancers and actinic keratosis is an essential pre requisite to establish new strategies to control these conditions. .

A queratose actínica é uma causa comum de consultas dermatológicas e apresenta forte associação com o carcinoma espinocelular. Muitas substâncias são utilizadas para seu tratamento e prevenção, assim como os retinoides. Entretanto, muitos estudos sobre retinoides salientam seu uso no tratamento e prevenção de cânceres de pele não melanoma. Neste artigo, nós revisamos estudos que avaliam o uso dos retinoides sistêmicos e tópicos para pacientes imunocompetentes e imunossuprimidos com queratoses actínicas, como desfechos primários e secundários. A maioria destes estudos mostra redução na contagem das queratoses actínicas após o tratamento com retinóides. Além disso, ajustes no delineamento dos estudos deveriam ser feitos quanto à falta de padronização da dose, ausência de grupos controle, número pequeno de pacientes e tempo curto de seguimento. Ensaios clínicos cegos, randomizados e controlados com tamanho amostral adequado tendo como alvo específico as queratoses actínicas são necessários para esclarecer o real benefício dos retinoides tópicos e/ou orais. A comparação da eficácia e segurança entre os retinoides orais e tópicos na prevenção e tratamento dos cânceres de pele não melanoma e queratoses actínicas é um pré-requisito essencial para o estabelecimento de novas estratégias para o controle destas condições.

Enfermedad de Darier tratada con acitretina oral / Darier's disease treated with oral acitretin

La enfermedad de Darier (ED) descrita por Darier y White en 1889, es un trastorno autosómico dominante de la queratinización, causada por una mutación del gen ATP2A2, localizado en el cromosoma 12 que codifica para la bomba de calcio ATP-asa de tipo 2 del retículo sarco-endoplásmico (SERCA-2), que conduce a una queratinización anormal (disqueratosis) y pérdida de la adhesión intercelular de los queratinocitos (acantolisis), con la consecuente formación de hendiduras suprabasales. Se caracteriza por alteraciones de la queratinización de la epidermis, uñas y mucosas. Presentamos el caso de un paciente masculino de 21 años, con lesiones cutáneas e histológicas características de (ED), quien realizó tratamiento con acitretina oral a una dosis de 30 mg al día, con respuesta favorable. Se plantea una alternativa terapéutica con retinoides orales para las lesiones crónicas y resistentes.

Darier's disease (DD) described by Darier and White in 1889 is an autosomic dominant disorder of keratinization, caused by a mutation of gen ATP2A2, located in the chromosome 12 encoding for calcium ATPase pump of sarcoplasmic reticulum type 2 (SERCA-2) leading to abnormal keratinization (dyskeratosis) and loss of intercellular adhesion of keratinocytes (acantholysis), with the consequent formation of suprabasal clefts. Characterized for abnormal keratinization in the epidermis, nails and mucosae. We present the case of a 21-year-old, male patient, with clinically and histologically confirmed (DD) treated with oral acitretin 30 mg for day with good response. We recommend an optional therapeutic treatment for chronic, itchy, recalcitrant lesions with oral retinoids.

Deficiencia de retinoides y consumo de estrógenos como cofactores de riesgo de cáncer cervical / Retinoid deficiency and estrogens as risk cofactors in cervical cancer

La infección persistente por el virus del papiloma humano de alto riesgo (HR-HPV) está relacionada con la aparición de cáncer cervical (CC), una de las principales causas de mortalidad por cáncer en todo el mundo. La infección se produce en la zona de transformación, la región más sensible del cérvix a estrógenos y retinoides. El CC afecta a un bajo porcentaje de mujeres infectadas por HR-HPV y tarda en desarrollarse hasta décadas después de la infección, lo que sugiere que el HR-HPV es necesario pero no suficiente para causar CC. Otros factores son necesarios para la progresión desde la infección por HR-HPV hasta el cáncer, como por ejemplo: uso de anticonceptivos orales por largos períodos, fumar, partos múltiples, falta de micronutrientes, particularmente una dieta baja en retinoides, los cuales alteran la diferenciación epitelial, el crecimiento celular y la apoptosis de las células malignas. La detección precoz del HR-HPV y el manejo de lesiones precancerosas, aunado a un conocimiento detallado de factores de riesgo adicionales, puede ser una estrategia para prevenir esta enfermedad. La presente revisión se enfoca en explicar el efecto de los estrógenos, la deficiencia de retinoides y el HR-HPV en la aparición del CC. Dichos cofactores pueden actuar en conjunto para inducir transformación neoplásica en el epitelio escamoso del cérvix, promoviendo un segundo evento genético o epigenético que lleve a la aparición del CC.

ersistent infection with high-risk human papillomaviruses (HR-HPVs) is involved in cervical cancer (CC),a major cause of cancer mortality worldwide. Infection occurs primarily at the transformation zone (TZ),the most estrogen- and retinoid-sensitive region of the cervix. Development of CC affects a small per-centage of HR-HPV-infected women and often takes decades after infection, suggesting that HR-HPVis a necessary but not sufficient cause of CC. Thus, other cofactors are necessary for progression fromcervical HR-HPV infection to cancer such as long-term use of hormonal contraceptives, multiparity, smo-king, as well as micronutrient depletion and in particular retinoid deficiency, which alters epithelial diffe-rentiation, cellular growth and apoptosis of malignant cells. Therefore, early detection of HR-HPV andmanagement of precancerous lesions together with a profound understanding of additional risk factorscould be a strategy to avoid this disease. In this review we focus on the synergic effect of estrogens,retinoid deficiency and HR-HPVs in the development of CC. These risk factors may act in concert to indu-ce neoplastic transformation in the squamous epithelium of the cervix, setting the stage for secondarygenetic or epigenetic events leading to cervical cancer.

Avaliação de dermocosmético com retinaldeído, nicotinamida e vitis vinifera no fotoenvelhecimento cutâneo de mulheres entre 25 e 40 anos de idade / Assessment of dermocosmetics contai- ning retinaldehyde, nicotinamide and Vitis vinifera in the cutaneous photoaging of women between 25 and 40 years old

Introdução: A pele está sujeita ao envelhecimento intrínseco e extrínseco. Desse modo, tratar o fotoenvelhecimento cutâneo representa desafio clínico. Objetivo: Avaliar, através de parâmetros objetivos e subjetivos, segurança, tolerabilidade e eficácia clínica de produto cosmético contendo retinaldeído 0,05%, nicotinamina 4% e extrato de Vitis vinifera 1% no tratamento do envelhecimento da pele. Métodos: Estudo monocêntrico, prospectivo, intervencional, aberto que avaliou 40 voluntárias do sexo feminino de 25 a 40 anos, fototipos I a IV de Fitzpatrick, que aplicaram produto dermocosmético durante 60 dias consecutivos. As avaliações foram realizadas de forma subjetiva pelo médico, voluntária e exameultrassonográfico e de forma objetiva por fotografias pelo Visia®, biópsias cutâneas, corneometria, pHmetria e sebumetria. Resultados: 36 voluntárias completaram o estudo. Avaliações subjetivas demonstraram melhora nas rugas, linhas finas, flacidez, viço e aparência geral. Ultrassom e biópsia cutânea evidenciaram aumento de colágeno em 94,4% e 30,6% respectivamente. Fotos do aparelho Visia® demonstraram redução de manchas, rugas, poros e melhora da textura cutânea. Sebumeter revelou diminuição da oleosidade, e houve redução do pH. As medidas do Corneometer mantiveram-se constantes. Conclusões: o produto dermocosmético analisado foi eficaz e seguro para combater o fotoenvelhecimento.

Acne fulminans e isotretinoína: relato de caso / Acne fulminans and isotretinoin: case report

A acne fulminans é afecção rara e a forma mais grave de todo o espectro clínico da acne. Caracterizase pelo aparecimento súbito de nódulos inflamatórios dolorosos que ulceram, concomitantemente, a manifestações sistêmicas. Pode ser induzida pela isotretinoína e alguns autores acreditam que a ocorrência do quadro seja dose dependente. O tratamento é controverso, não havendo padronização até o momento. Apresenta-se caso desta rara doença, desenvolvida durante o uso de isotretinoína em baixas doses.

Acne fulminans (AF) is a rare disorder, the most severe form of the entire clinical spectrum of acne. It is characterized by emergence of painful inflammatory nodules that turn into ulcers, concomitant with systemic manifestations. It can be induced by isotretinoin and some authors believe that the occurrence of this condition is dose dependent. The treatment is controversial and there is no consensus up to this time. A case of this rare disease, developed during use of isotretinoin in low doses, is presented.

Hiperceratose epidermolítica: um seguimento de 23 anos de uso de retinoides orais / Epidermolytic hyperkeratosis: a follow-up of 23 years of use of systemic retinoids

A hiperceratose epidermolítica é uma forma de ictiose geralmente resistente a tratamentos tópicos. Relata-se um caso de paciente feminina , em acompanhamento na dermatologia desde 1978, com diagnóstico de hiperceratose epidermolítica. Foi tratada inicialmente com queratolíticos, vitamina A oral, ácido tartárico e emolientes tópicos, porém sem melhora no quadro clínico, já que não haviam disponíveis outros tratamentos na época. Em 1986, com o advento dos retinóides orais, foi introduzido o etretinato, e em 1998, foi substituído pelo acitretin, apresentando excelente resposta terapêutica. No momento a paciente está em uso de acitretin 25 mg/dia, completando 23 anos de uso de retinóides orais, com mínimos efeitos adversos e melhora significativa na qualidade de vida.

Epidermolytic hyperkeratosis is a form of ichthyosis normally resistant to topical treatments. Female patient monitored since 1978 diagnosed with epidermolytic hyperkeratosis. Clinical examination showed generalized hyperkeratosis and scaling. Given that no other treatments were available at the time, the patient was initially treated with keratolytic, systemic vitamin A and moisturizers, with no improvement. In 1986, with the development of oral retinoids, etretinate was introduced. In 1998 this was replaced by acitretin. The patient is receiving 25 mg/day after 23 years of using oral retinoids. Significant improvement of the condition and patient's quality of life has been noted.

Mulheres adultas com acne apresentam maior risco de elevação de triglicerídeos ao uso de isotretinoína oral / Adult women with acne have a higher risk of elevated triglyceride levels with the use of oral isotretinoin

A isotretinoína apresenta restrições relacionadas a efeitos no perfil lipídico. Revisaram-se 90 pacientes tratados, em busca de fatores predisponentes a essas alterações. Houve elevação significativa do colesterol e triglicerídeos. Os pacientes em que estes últimos mostraram essa alteração foram, em sua maioria, do sexo feminino, predileção que não ocorria com as alterações iniciais. Mulheres com acne persistente talvez representem população de risco para tais efeitos colaterais.

There are restrictions associated with the use of isotretinoin because of its effects on the lipid profile. The records of ninety patients treated with this medication were reviewed to identify factors that would predispose patients to these abnormalities. A significant increase in cholesterol and triglyceride levels occurred. Patients in whom triglyceride levels increased were more likely to be female, although this difference was not present at baseline. Women with persistent acne may constitute a risk population for these side effects.

Enfermedad de Darier-White tratada con isotretinoína oral / Darier-White disease treated with oral isotretinoin

La enfermedad de Darier-White (EDW) es un trastorno autosómico dominante; descrita por Darier y White en 1889, se caracteriza por alteraciones de la queratinización de la epidermis, uñas y mucosas. Presentamos el caso de un paciente masculino de 16 años con lesiones cutáneas e histológicas características de la EDW, que realizó tratamiento con isotretinoína oral a una dosis de 0,7 mg/kg/día por seis meses, con respuesta favorable. Se plantea una alternativa terapéutica con retinoides orales para las lesiones fjas, resistentes, crónicas y pruriginosas.

Darier-White Disease (DWD) is an autosomal dominant disorder, described by Darier and White in 1889, characterized by abnormal keratinization in the epidermis, nails and mucosae. We present the case of a 16-year-old, male patient, with clinically and histologically confrmed DWD treated with oral isotretinoin (0.7 mg/kg/day) for six months, with good response. We recommend an optional therapeutic treatment for chronic, itchy, recalcitrant lesions with oral retinoids.

Ichthyosis follicularis, alopecia and photophobia syndrome (IFAP): report of the first case with ocular and cutaneous manifestations in Brazil with a favorable response to treatment / Síndrome de ictiose folicular, alopécia e fotofobia (IFAP): relato do primeiro caso com acometimento ocular e cutâneo do Brasil e da resposta favorável ao tratamento

Ichthyosis follicular, alopecia, and photophobia (IFAP) syndrome is a rare disease, with possible X-linked mode of inheritance. The patient presented with ocular findings of photophobia, corneal scarring and erosions, superficial and deep corneal vascularization and myopia. He was treated with artificial tears and punctal occlusion with small improvement of photophobia. After three months using systemic retinoid (Acitretina) and posterior amniotic membrane transplantation in the left eye, there was a significant improvement of photophobia, corneal erosions and neuropsychomotor development.

A síndrome de ictiose folicular, alopecia e fotofobia (IFAP) é uma doença rara, com possível modo de herança ligado ao cromossomo X. O paciente apresentou achados oftalmológicos de fotofobia, cicatrizes e erosão corneanas, neovascularização superficial e profunda da córnea e miopia. Foi iniciado uso de lubrificantes e oclusão do ponto lacrimal com discreta melhora da fotofobia. Após uso de retinóide sistêmico (Acitretina) por três meses e posterior transplante de membrana amniótica no olho esquerdo apresentou melhora importante da fotofobia, das erosões corneanas e do desenvolvimento neuropsicomotor.

Acne da mulher adulta: aspectos epidemiológicos, diagnósticos e terapêuticos / Acne in adult women: epidemiological, diagnostic and therapeutic aspects

FUNDAMENTOS: A acne da mulher adulta (AMA) é uma dermatose de incidência crescente; o hiperandrogenismo está presente em muitos casos, mas há pacientes sem anormalidades hormonais. OBJETIVO: Analisar as características prevalentes na mulher adulta com acne sem hiperandrogenismo. MATERIAL E MÉTODO: Um levantamento de 226 prontuários com queixa de AMA foi realizado; 116 pacientes (51,3 por cento) possuíam perfil hormonal normal e, portanto, foram incluídas no estudo. Foram pesquisados idade, etnia, perfil clínico da acne, exames complementares, tratamentos anteriores, tratamentos prescritos e evolução. RESULTADOS: A média das idades foi de 33,9 anos e o grau clínico predominante foi o inflamatório moderado (grau II); a face foi a área mais acometida. Os retinoides tópicos foram as drogas mais prescritas, e a medicação sistêmica foi indicada em 53,4 por cento das pacientes. A regressão total foi observada em 31 pacientes (26,7 por cento) até 12 semanas de tratamento. Houve reação adversa ao tratamento tópico em 21,5 por cento das pacientes. CONCLUSÃO: O quadro clínico da AMA em pacientes sem hiperandrogenismo é de moderada intensidade, com predomínio de lesões inflamatórias. Seu tratamento é similar ao da acne vulgar; entretanto, atenção especial deve ser tomada com medicações de maior potencial irritante, uma vez que esse grupo parece ter uma maior predisposição a irritações cutâneas.

BACKGROUND: Adult female acne (AFA) is a dermatosis of increasing incidence; hyperandrogenism is present in many cases, but there are patients without hormonal abnormalities. OBJECTIVE: To analyze the prevalent characteristics in adult women with acne without hyperandrogenism. MATERIAL AND METHODS: A survey of 226 medical records with AFA complaints was conducted; of these, 116 (51.3 percent) had a normal hormonal profile and were, therefore, included in the study. We investigated age, ethnicity, clinical profile of acne, complementary exams, previous treatment, prescribed treatment and evolution. RESULTS: The mean age was 33.9 years and the predominant clinical grade of acne was moderate inflammatory (grade 2). The face was the most affected area. Topical retinoids were the most prescribed drugs, and systemic medication was prescribed to 53.4 percent of the patients. Total regression was observed in 31 patients (26.7 percent) within 12 weeks of treatment. Adverse reactions to topical treatment occurred in 21.5 percent of the patients. CONCLUSION: The clinical manifestations of AFA in patients without hyperandrogenism are moderate, with predominance of inflammatory lesions. Treatment is similar to that of acne vulgaris; however, special attention should be taken with more irritating medication since this group appears to be more predisposed to skin irritations.