Manejo de reações adversas dermatológicas em pacientes submetidos à terapia quimioterápica antineoplásica / Management of dermatological adverse reactions in patients undergoing antineoplastic chemotherapy

Objetivo: o presente estudo tem como objetivo reunir recomendações de cuidados considerando a prevenção e tratamento de lesões de pele induzidas pelo tratamento com quimioterápicos antineoplásicos, de acordo com os estudos e consensos atuais. Metodologia: realizou-se um estudo bibliográfico para levantamento das relações entre os principais fármacos antineoplásicos e suas intercorrências dermatológicas, bem como seus respectivos manejos, para subsidiar a orientação e aconselhamento aos profissionais de saúde que acompanham o paciente oncológico. Resultado: os principais problemas dermatológicos decorrentes do uso de antineoplásicos correspondem às lesões de pele, tais como a descoloração, hiperpigmentação, fotossensibilidade, eritemas, descamação e prurido. Também são recorrentes os efeitos adversos que acometem os pelos e cabelos, resultando em alopecia, e a modificação do crescimento e lesões nas unhas. Tratamentos específicos para cada caso são capazes de amenizar ou reverter os problemas. Conclusão: as reações adversas aos medicamentos envolvendo quimioterapia são frequentes na prática oncológica, e variam em termos de frequência e gravidade, atingindo diversos anexos cutâneos. O adequado manejo destes efeitos melhora a integridade da pele e demais estruturas, proporcionando a esses pacientes a melhoria da autoestima e da qualidade de vida.

Objective: the present study aims to gather care recommendations considering the prevention and treatment of skin lesions induced by treatment with antineoplastic chemotherapy, according to current studies and consensus. Methodology: a bibliographical study was carried out to survey the relationships between the main antineoplastic drugs and their dermatological intercurrences, as well as their respective management, to subsidize the guidance and counselling of health professionals who treat cancer patients. Result: the main dermatological problems arising from the use of antineoplastic agents correspond to skin lesions, such as discoloration, hyperpigmentation, photosensitivity, erythema, scaling and pruritus. Adverse effects that affect hair and body hair are also recurrent, resulting in alopecia, and the modification of growth and lesions on the nails. Specific treatments for each case can alleviate or reverse the problems. Conclusion: adverse drug reactions involving chemotherapy are frequent in oncology practice, and vary in terms of frequency and severity, affecting various skin appendages. Proper management of these effects improves the integrity of the skin and other structures, providing these patients with improved self-esteem and quality of life.

Papilomatosis confluente y reticulada de Gougerot-Carteaud / CONFLUENT AND RETICULATED PAPILLOMATOSIS OF GOUGEROT-CARTAUD

La papilomatosis confluente y reticulada de Gougerot-Cartaud es una dermatosis poco frecuente, de etiología incierta. Afecta a adolescentes y adultos jóvenes, con leve predilección por el sexo masculino. Se presenta como pápulas parduzcas que confluyen formando placas centrales con patrón reticulado en la periferia, asintomáticas o levemente pruriginosas. La localización característica es tronco anterior y posterior, a nivel de la línea media. Los antibióticos orales, principalmente la minociclina, constituyen el tratamiento de elección. Debe diferenciarse de otras entidades, entre ellas pitiriasis versicolor, acantosis nigricans y dermatosis terra firma-forme. Se presenta una paciente de 17 años con papilomatosis confluente y reticulada de GougerotCarteaud que respondió satisfactoriamente al tratamiento con minociclina vía oral y tretinoína 0,025% tópica.

Confluent and reticulated papillomatosis of Gougerot-Cartaud is a rare dermatosis, of still uncertain etiology. It affects adolescents and young adults, with a slight predilection for males. It presents as asymptomatic or slightly pruritic brownish papules that converge to form central plaques with a reticulated pattern on the periphery. The characteristic location is midline anterior and posterior trunk. Oral antibiotics, mainly minocycline, are the treatment of choice. It must be differentiated from other entities, including pityriasis versicolor, acanthosis nigricans, and terra firme-forme dermatosis. We present a 17-year-old patient with confluent Gougerot-Carteaud papillomatosis who responded satisfactorily to treatment with oral minocycline and topical tretinoin 0.025%.

Fibrosis pulmonar inducida por metotrexato en un paciente con psoriasis / Methotrexate-induced pulmonary fibrosis in a patient with psoriasis

La fibrosis pulmonar a causa del metotrexato es un efecto adverso infrecuente, observado principalmente en los pacientes con artritis reumatoide, aunque también se vio, de manera escasa, en el tratamiento de la psoriasis. Se presenta el caso de un paciente con psoriasis que desarrolló fibrosis pulmonar por metotrexato.

Pulmonary fibrosis due to methotrexate is an infrequent adverse event, observed mainly in patients with rheumatoid arthritis, although it has also been poorly described in the treatment of psoriasis. We present the case of a patient with psoriasis who developed pulmonary fibrosis due to methotrexate.

Psoriasis paradójica palmoplantar secundaria al tratamiento de la colitis ulcerosa con adalimumab / Paradoxical psoriasis of the palms and soles due to treatment of ulcerative colitis with adalimumab

Los anticuerpos anti-TNF-a (tumor necrosis factor alpha) se utilizan para tratar tanto la psoriasis como la enfermedad inflamatoria intestinal (EII). Sin embargo, estos fármacos han sido implicados en la ocurrencia de la psoriasis paradójica en los pacientes sin antecedentes de psoriasis que reciben tratamiento por una colitis ulcerosa (CU) y otras enfermedades autoinmunes. Se presenta el caso de un paciente de 29 años, sin antecedentes de dermatosis, que desarrolló una psoriasis palmoplantar paradójica por el uso del adalimumab que recibía por un diagnóstico de CU. El cuadro remitió al suspender el medicamento y recurrió al reiniciarlo, motivo por el cual se rotó al ustekinumab. La CU respondió satisfactoriamente, sin nuevas lesiones dermatológicas.

Anti TNF-a (tumor necrosis factor alpha) antibodies are used to treat both psoriasis and inflammatory bowel disease (IBD). However, these drugs have been implicated in the occurrence of the so-called paradoxical psoriasis in patients with no previous history of psoriasis, who receive treatment for ulcerative colitis and other autoimmune diseases. We present a 29-year-old male patient, with no previous history of dermatosis, who developed paradoxical palmar-plantar psoriasis due to the use of adalimumab that he was receiving for a diagnosis of ulcerative colitis. The condition remitted when the drug was suspended and recurred when it was restarted, and for that reason, treatment was rotated to ustekinumab. Ulcerative colitis responded satisfactorily, with no new dermatological lesions.

Presente y futuro del tratamiento biológico y moléculas pequeñas en la dermatitis atópica / Present and future of biological treatment and small molecules in atopic dermatitis

La dermatitis atópica (DA) es una condición inflamatoria crónica de la piel de etiología multifactorial. Buscando mejorar la respuesta clínica minimizando los efectos adversos y ampliar el arsenal terapéutico disponible, se ha dado pie al desarrollo de nuevos fármacos con resultados prometedores en la calidad de vida. Los inmunomoduladores sistémicos clásicos son considerados el tratamiento estándar en los casos de DA moderada a severa refractaria al tratamiento con corticoides tópicos. Estos se encasillan dentro de las denominadas moléculas pequeñas, junto con los inhibidores de Janus- en un efecto pleiotrópico en las citoquinas y por ende, no selectivo. Los medicamentos biológicos poseen ventajas frente a los inmunomoduladores clásicos, principalmente su mayor especificidad gracias a la similitud con las moléculas endógenas. Dupilumab se mantiene siendo el único fármaco biológico aprobado por la FDA para el tratamiento de la DA, con una seguridad a corto plazo demostrada. Algunas moléculas nuevas, como el tralokinumab y los inhibidores JAK, presentan resultados prometedores. De este grupo, abrocitinib pareciera posicionarse como una alternativa al menos similar que dupilumab. La creciente investigación de nuevas alternativas ha creado una revolución terapéutica para que nuestros pacientes puedan acceder a una mejor calidad de vida. No obstante, es difícil lograr comprender la efectividad y seguridad de cada uno de los tratamientos disponibles, por la falta de estudios comparativos. La siguiente revisión muestra las nuevas terapias biológicas y algunas moléculas pequeñas con evidencia para su uso en DA

Atopic dermatitis (AD) is a chronic inflammatory condition of the skin with a multifactorial etiology. Seeking to improve the clinical response by minimizing adverse effects and expanding the available therapeutic arsenal, the development of new drugs has led to promising results on quality of life. Classic systemic immunomodulators are considered the standard treatment in cases of moderate to severe AD refractory to treatment with topical corticosteroids. These are classified into molecules, along with Janus kinase inhibitors (JAKs). Small molecules act on intracellular targets, with the inconveniency of producing a pleiotropic effect on cytokines and, therefore, non-selective actions. Biologics have advantages over classical immunomodulators, mainly their greater specificity thanks to the similarity between endogenous molecules. Dupilumab remains the only biologic drug approved by the FDA for the treatment of AD, with demonstrated short-term safety. Some new molecules, such as tralokinumab and JAK inhibitors, have shown promising results. Of this group, abrocitinib seems to be positioned as an alternative at least similar to dupilumab. The current investigation of new alternatives has created a therapeutic revolution so that we can offer our patients a better quality of life. However, it is difficult to understand the efficacy and safety of each of the available treatments due to the lack of comparative studies. The following review shows the new biological therapies and small molecules with evidence for their use in DA.

Consensus on the use of oral isotretinoin in dermatology - Brazilian Society of Dermatology

Abstract Background: Isotretinoin is a synthetic retinoid, derived from vitamin A, with multiple mechanisms of action and highly effective in the treatment of acne, despite common adverse events, manageable and dose-dependent. Dose-independent teratogenicity is the most serious. Therefore, off-label prescriptions require strict criteria. Objective: To communicate the experience and recommendation of Brazilian dermatologists on oral use of the drug in dermatology. Methods: Eight experts from five universities were appointed by the Brazilian Society of Dermatology to develop a consensus on indications for this drug. Through the adapted DELPHI methodology, relevant elements were listed and an extensive analysis of the literature was carried out. The consensus was defined with the approval of at least 70% of the experts. Results: With 100% approval from the authors, there was no doubt about the efficacy of oral isotretinoin in the treatment of acne, including as an adjunct in the correction of scars. Common and manageable common adverse events are mucocutaneous in nature. Others, such as growth retardation, abnormal healing, depression, and inflammatory bowel disease have been thoroughly investigated, and there is no evidence of a causal association; they are rare, individual, and should not contraindicate the use of the drug. Regarding unapproved indications, it may represent an option in cases of refractory rosacea, severe seborrheic dermatitis, stabilization of field cancerization with advanced photoaging and, although incipient, frontal fibrosing alopecia. For keratinization disorders, acitretin performs better. In the opinion of the authors, indications for purely esthetic purposes or oil control are not recommended, particularly for women of childbearing age. Conclusions: Approved and non-approved indications, efficacy and adverse effects of oral isotretinoin in dermatology were presented and critically evaluated.

A randomized half-body, double blind, controlled trial on the effects of a pH-modified moisturizer vs. standard moisturizer in mild to moderate atopic dermatitis,

Abstract Background: Higher skin pH in atopic dermatitis contributes to impaired epidermal barrier. A moisturizer compatible with physiological pH could improve atopic dermatitis. Objective: To determine the effect of a physiologically compatible pH moisturizer in atopic dermatitis. Methods: A randomized half body, double blind, controlled trial involving patients with stable atopic dermatitis was performed. pH-modified moisturizer and standard moisturizer were applied to half body for 6 weeks. Results: A total of 6 (16.7%) males and 30 (83.3%) females participated. Skin pH reductions from week 0, week 2 and 6 were significant at the forearms (5.315 [0.98] to 4.85 [0.54] to 5.04 [0.78], p = 0.02) and abdomen (5.25 [1.01], 4.82 [0.64], 5.01 [0.59], p = 0.00) but not at the shins (5.01 [0.80], 4.76 [0.49], 4.85 [0.79], p = 0.09) with pH-modified moisturizer. Transepidermal water loss (TEWL) at the forearms decreased (4.60 [2.55] to 3.70 [3.10] to 3.00 [3.55], p = 0.00), abdomen (3.90 [2.90] to 2.40 [3.45] to 2.70 [2.25], p = 0.046). SCORAD improved from 14.1 ± 12.75 to 10.5 ± 13.25 to 7 ± 12.25, p = 0.00. In standard moisturizer group, pH reductions were significant at the forearms (5.29 [0.94] to 4.84 [0.55] to 5.02 [0.70], p = 0.00) and abdomen (5.25 [1.09], 4.91 [0.63], 5.12 [0.66], p = 0.00). TEWL at the forearm were (4.80 [2.95], 4.10 [2.15], 4.60 [3.40], p = 0.67), shins (3.80 [1.40], 3.50 [2.35], 4.00 [2.50], p = 0.91) and abdomen (3.70 [2.45], 4.10 [3.60], 3.40 [2.95], p = 0.80). SCORAD improved from 14.2 ± 9.1 to 10.9 ± 10.65 to 10.5 ± 11, p = 0.00. Reduction in pH was observed with both moisturizers while TEWL significantly improved with pH-modified moisturizer. pH-modified moisturizer resulted in greater pH, TEWL and SCORAD improvements however the differences were not significant from standard moisturizer. Study limitation: Skin hydration was not evaluated. Conclusion: Moisturization is beneficial for atopic dermatitis; use of physiologically compatible pH moisturizer is promising.

Ocular surface changes in the treatment of rosacea: comparison between low-dose oral isotretinoin and doxycycline / Alterações da superfície ocular no tratamento da Rosácea: comparação entre isotretinoína oral em baixa dosagem e doxiciclina

ABSTRACT Purpose: To compare the impact of ocular changes between systemic treatment with doxycycline and low-dose oral isotretinoin in patients with moderate-to-severe papulopustular rosacea. Methods: Patients were randomized to receive either isotretinoin 0.3-0.4 mg/kg (group A) or doxycycline 100 mg/day (group B) for 16 weeks. Ocular symptoms were searched and evaluated, including best-corrected visual acuity (BCVA), Schirmer test, breakup time, rose bengal staining score, and meibomian gland dysfunction grading. The patients were retested at the end of treatment. Results: The present study included 39 patients (30 females and 9 males). Best-corrected visual acuity was > 20/30 in >90% of patients in both groups and did not change after treatment. After treatment, improvement in ocular symptoms and meibomian gland dysfunction was more pronounced in group B (p<0.05); the other parameters did not reach statistical significance. Conclusion: Doxycycline improved meibomian gland dysfunction, ocular symptoms, and ocular surface in patients with rosacea. Even though some patients experienced worsening meibomian gland dysfunction and symptoms, no subject experienced any serious complications after administration of low-dose isotretinoin.

RESUMO Objetivos: Comparar o impacto das alterações oculares entre o tratamento sistêmico de doxiciclina e isotretinoína em baixa dosagem em pacientes com rosácea papulopustulosa moderada a grave. Métodos: Os pacientes form randomizados para receber isotretinoína 0,3 a 0,4 mg/kg (grupo A) ou doxiciclina 100mg/dia (grupo B) por 16 semanas. Os sintomas oculares foram pesquisados e avaliados, incluindo melhor acuidade visual corrigida, teste de Schirmer, tempo de ruptura do filme lacrimal, coloração de rosa bengala e graduação da disfunção de glândula de Meibomius. Os pacientes foram novamente testados no final do tratamento. Resultados: O presente estudo incluiu 39 pacientes (30 mulheres e 9 homens). A melhor acuidade visual corrigida foi >20/30 em >90% dos pacientes em ambos os grupos e não se alterou após o tratamento. A melhora dos sintomas oculares e da disfunção de glândula de Meibomius foi mais pronunciada no grupo B (p<0,05) após o tratamento; as demais variáveis não atingiram significância estatística. Conclusão: A doxiciclina melhorou a disfunção de glândula de Meibomius, os sintomas oculares e a superfície ocular de pa cientes com rosácea. Mesmo que alguns pacientes tenham piorado a disfunção e os sintomas da glândula de Meibomius, nenhum indivíduo apresentou complicações graves após a admi nistração de baixas doses de isotretinoína.

Analysis of cardiovascular risk and carotid intima-media thickness in patients with psoriasis

Abstract Background: Psoriasis is associated with atherosclerosis and increased cardiovascular risk. Currently, an automated ultrasound, called quantitative intima media thickness, has proven to be a useful method to evaluate subclinical atherosclerosis. Objectives: To compare increased cardiovascular risk in psoriasis patients receiving two types of treatments: Methotrexate and tumor necrosis factor inhibitor and to evaluate the correlation between the Framingham score and quantitative intima media thickness. Methods: Fifty patients with plaque psoriasis were selected from June 2017 to July 2018, divided into two groups, receiving methotrexate and tumor necrosis factor inhibitor. Measurement of abdominal circumference, blood pressure, body mass index and presence of metabolic syndrome were performed. Afterwards, the patients were evaluated for increased cardiovascular risk with the Framingham score and for the quantitative intima media thickness of the carotid arteries. Results: The mean age was 54.8 (±12.5) with a slight male predominance (58%). Overall, 84% of the patients had elevated waist circumference, 82% had a body mass index above ideal, and 50% had a metabolic syndrome. For the correlation between quantitative intima media thickness and Framingham Score, Pearson's linear correlation coefficient was 0.617 (p < 0.001), indicating a moderate to strong positive association. Study limitations: The protective effect of the therapies cited in relation to the increased cardiovascular risk was not evaluated. Conclusions: A moderate to strong positive association was found correlating the Framingham Score values with the quantitative intima media thickness measurement and it is not possible to state which drug has the highest increased cardiovascular risk.

Effects of oral isotretinoin therapy on the nasal cavities / Efeitos nasais da terapia com isotretinoína oral

Abstract Introduction Isotretinoin (13 cis-retinoic acid) is the most effective treatment for acne vulgaris and is the only treatment option that can provide either remission or a permanent cure. Objective The aim of this study was to use both subjective and objective methods to assess the nasal complaints of patients with severe acne who received oral isotretinoin therapy. Methods Fifty-four subjects were enrolled in the study. All the subjects were assessed with subjective (NOSE and VAS questionnaires) and objective (rhinomanometry and saccharine) tests to determine the severity of their nasal complaints. Results The mean severity scores (min: 0; max: 100) for nasal dryness/crusting and epistaxis were 0.47 ± 1.48 (0-5); 0.35 ± 1.30 (0-5) at admission, 3.57 ± 4.45 (0-10); 2.26 ± 4.71 (0-20) at the first month, and 4.28 ± 6 (0-20); 2.26 ± 4.71 (0-20) at the third month of the treatment respectively. Total nasal resistance of 0.195 ± 0.079 (0.12-0.56) Pa/cm3/s at admission, 0.21 ± 0.084 (0.12-0.54) Pa/cm3/s at the first month, and 0.216 ± 0.081 (0.14-0.54) Pa/cm3/s at the third month. Conclusion Oral isotretinoin therapy can cause the complaint of nasal obstruction. In addition, nasal complaints, such as dryness/crusting and epistaxis, significantly increase in patients during the therapy schedule.

Resumo Introdução A isotretinoína (ácido-13 cis-retinóico) é o tratamento por via oral mais eficaz para acne vulgar e é a única opção de tratamento que pode produzir remissão ou cura permanente. Objetivo Usar métodos subjetivos e objetivos para avaliar as queixas nasais de pacientes com acne grave que receberam terapia com isotretinoína oral. Método Foram incluídos no estudo 54 indivíduos. Todos os indivíduos foram avaliados por meio de testes subjetivos (questionários NOSE e escala EVA) e objetivos (rinomanometria e teste de sacarina) para determinar a gravidade de suas queixas nasais. Resultados Os escores médios de gravidade (min: 0; max: 100) para ressecamento/crostas e epistaxe nasal foram de 0,47 ± 1,48 (0-5); 0,35 ± 1,30 (0-5) no início, 3,57 ± 4,45 (0-10); 2,26 ± 4,71 (0-20) no primeiro mês e 4,28 ± 6 (0-20); 2,26 ± 4,71 (0-20) no terceiro mês do tratamento, respectivamente. A resistência nasal total foi de 0,195 ± 0,079 (0,12 a 0,56) Pa/cm3/s no início, 0,21 ± 0,084 (0,12 a 0,54) Pa/cm3/s no primeiro mês e 0,216 ± 0,081 (0,14 a 0,54) Pa/cm3/s no terceiro mês. Conclusão A terapia com isotretinoína por via oral pode resultar em queixa de obstrução nasal. Além disso, queixas nasais, tais como ressecamento/formação de crostas e epistaxe, aumentam significativamente nos pacientes durante o esquema terapêutico.

Cutaneous sarcoidosis: clinico-epidemiological profile of 72 patients at a tertiary hospital in São Paulo, Brazil,

Abstract Background: Sarcoidosis is a multisystem disease of unknown cause that is characterized by the presence of granulomas in various organs. Cutaneous involvement is common and the reported incidence has varied from 9% to 37%. Studies on cutaneous sarcoidosis in Brazil are lacking. Objectives: To describe the clinical and epidemiological aspects of patients with cutaneous sarcoidosis diagnosed at the Department of Dermatology of the University of São Paulo, from May 1994 to March 2018. Methods: Clinical data of patients with confirmed cutaneous sarcoidosis were retrospectively reviewed and classified according to gender, ethnicity, age at diagnosis, cutaneous presentation, systemic involvement and treatment. Results: Cutaneous sarcoidosis was diagnosed in 72 patients with a female predominance (74%). The mean age at diagnosis was 49.6 years and most of the patients were white (61%). Papules and plaques were the most common lesions. Systemic sarcoidosis was detected in 81% of patients, affecting mainly the lungs and thoracic lymph nodes (97%). Typically, cutaneous lesions were the first manifestation (74%). Systemic therapy was necessary for 72% of patients; the dermatologist managed many of these cases. Oral glucocorticoids were the most commonly used systemic medication (92%). The mean number of systemic drugs used was 1.98 per patient. Limitations: Insufficient data in medical records. Conclusions: This series highlights the dermatologist role in recognizing and diagnosing cutaneous sarcoidosis, evaluating patients for systemic disease involvement and treating the skin manifestations. Cutaneous sarcoidosis was once considered exceedingly infrequent in Brazil in comparison to infectious granulomatous diseases; however, the present series seems to suggest that the disease is not so rare in this region.

Acantholytic pityriasis rubra pilaris associated with topical use of imiquimod 5%: case report and literature review

Abstract Topical use of immune response modifiers, such as imiquimod, has increased in dermatology. Although its topical use is well tolerated, it may be associated with exacerbations of generalized cutaneous inflammatory diseases, possibly through the systemic circulation of pro-inflammatory cytokines. This report describes a case of development of pityriasis rubra pilaris, a rare erythematous-papulosquamous dermatosis, in a woman aged 60 years during treatment with imiquimod 5% cream for actinic keratosis. It evolved with erythrodermic conditions and palmoplantar keratoderma, presenting progressive clinical resolution after the introduction of methotrexate. The authors emphasize the importance of recognizing possible systemic reactions associated with the topical use of imiquimod.

Profile of depigmenting cosmetics and dermatological products on the market

The skin is the largest and most exposed organ of the human body, therefore subject to diseases and alteration of its appearance. Among these alterations, the cutaneous hyperchromia may be cited. Currently, the market offers numerous products with depigmenting action to the treatment of such disorders. The aim of this work was to analyze depigmenting products commercialized in establishments in the city of Bento Gonçalves (RS, Brazil) and websites of cosmetic companies. It was found 45 products with depigmenting action and, from these, 59 different active agents were identified. The main active compounds found were kojic acid, arbutin, ascorbic acid, hydroquinone and glycolic acid. Another observed data was that in 78% of the studied products the active substances were being used in combination. The most used vehicles were also studied as a reference to the use of sunscreen in the treatment of cutaneous hyperchromia. The present work had identified in the market a variety of products with depigmentation action and, because of this, it aims to serve as a reference to the healthcare professionals, especially at the prescribing moment, looking for the best results, with regards to treatment efficiency and safety.(AU)

Morfeas con manifestaciones cefálicas / Morpheas with cephalic manifestations

Introducción: La hemiatrofia facial progresiva (HFP) o síndrome de Parry-Romberg y la morfea en golpe de sable (MGS) forman parte de las morfeas lineales cefálicas. Son enfermedades inflamatorias crónicas de la piel y tejidos subyacentes, que se caracterizan por esclerosis y atrofia cutánea. Objetivos: Describir las características clínicas, manifestaciones asociadas, histología, laboratorio, estudios complementarios y tratamientos instaurados. Diseño: Estudio retrospectivo descriptivo. Materiales y métodos: Se revisaron las historias clínicas de pacientes con morfea evaluados en el Sector Colagenopatías desde julio de 2010 hasta diciembre de 2016. Resultados: De 56 pacientes, 11 cumplieron con los criterios de inclusión, 7 con diagnóstico de HFP, 2 con MGS y 2 con ambas patologías. El 64% fueron mujeres. Las manifestaciones extracutáneas se presentaron en el 64% de los casos. El tratamiento utilizado en todos los pacientes fue el metotrexato, asociado o no, al uso de corticosteroides sistémicos. Conclusiones: La mayoría de nuestros resultados concuerdan con la bibliografía consultada, excepto las manifestaciones asociadas. Destacamos el tratamiento asociado de metotrexato y pulsos de corticosteroides intravenosos con resultados satisfactorios y bien tolerado. (AU)

Introduction: Progressive facial hemiatrophy (PFH) or Parry-Romberg Syndrome and morphea en coup de sabre are cephalic linear morpheas. They are chronics inflammatories diseases of the skin and underlying tissues, characterized by cutaneous atrophy and sclerosis. Objectives: To describe clinical features, associated extracutaneous manifestations, histological and laboratory findings, imaging and diagnostic modalities and treatments established in patients with diagnosis of HFP, MGS, or both, evaluated in our Department. Design: Retrospective descriptive study. Materials and methods: We included medical histories of patients diagnosed with morphea evaluated in Collagenopathy Sector from July 2010 up to December 2016. Results: Of 56 patients, 11 met the inclusion criteria, 7 with diagnosis of PFH, 2 with morphea en coup de sabre and 2 with both pathologies. 64% were women. 64% showed extracutaneous manifestations. The treatment used in all of the patients was methotrexate, associated or not, with the use of systemic corticosteroids. Conclusions: Most of our results agree with the bibliography consulted, with the exception of the associated manifestations. We emphasize the associated treatment of methotrexate and intravenous corticosteroid pulses with satisfactory results and well tolerated. (AU)