Idiopathic aseptic facial granuloma: A retrospective study of 43 cases.

BACKGROUND: Idiopathic aseptic facial granuloma (IAFG) is an underrecognized pediatric skin disease, currently considered within the spectrum of rosacea. It usually manifests as a solitary, reddish, asymptomatic nodule on the cheek that resolves spontaneously. METHODS: Retrospective and descriptive observational study of 43 pediatric patients with a clinical diagnosis of IAFG, followed between 2004 and 2022, at two general hospitals in Argentina. RESULTS: IAFG predominated in girls (65%) and the average age of onset was about 6 years. A single asymptomatic nodule was seen in 79% of patients. The most common localization was the cheek (58%) followed by lower eyelids (41%). Family history of rosacea was present in 16% of patients. A concomitant diagnosis of rosacea and periorificial dermatitis was made in 14% and 9% of our population, respectively. Past or present history of chalazia was detected in 42% of the children. IAFG diagnosis was mainly clinical (88% of cases). Oral antibiotics were the most common indicated treatment (84%). Complete healing was achieved by the majority, but 18% of those with eyelid compromise healed with scars. CONCLUSIONS: IAFG is a benign pediatric condition that physicians should recognize in order to manage correctly. We herein refer to a particular morphologic aspect of IAFG lesions affecting the lower eyelids, where nodules adopt a linear distribution and have a higher probability of involute leaving a scar. Also, we consider that the concomitant findings of rosacea, periorificial dermatitis and chalazia in our patients, reinforce the consideration of IAFG within the spectrum of rosacea.

Actualización práctica de diagnóstico y tratamiento de la urticaria crónica / Practical update for the diagnosis and treatment of chronic urticaria

Resumen La urticaria es un patrón distintivo de respuesta inflamatoria de piel y/o mucosas caracterizada por la aparición súbita de ronchas evanescentes, angioedema o ambos, asociados a prurito. Las formas agudas son frecuentes y se limitan a brotes de menos de 6 sema nas; mientras que las crónicas tienen una prevalencia menor al 1%, mayor duración y pueden ser espontáneas o inducibles. Los mecanismos etiopatogénicos involucrados en esta enfermedad incluyen la autoalergia, la autoinmunidad y la inflamación con la activación celular, principalmente del mastocito, lo que lleva a su degranulación con libe ración de mediadores vasoactivos. En su abordaje son fundamentales la confirmación diagnóstica; la búsqueda de indicadores de su etiopa togenia; la detección de cofactores que pueden modular su actividad; el reconocimiento de comorbilidades; la evaluación de posibles biomarcadores y, el impacto en la calidad de vida, el registro de la actividad y el control de la enfermedad. El manejo farmacológico tiene por objetivo controlar los síntomas, mientras la urticaria resuelve de forma espontánea. Este se describe de forma escalonada con una complejidad creciente.

Abstract Urticaria is a distinctive pattern of inflammatory re sponse of the skin and/or mucous membranes charac terized by the sudden appearance of vanishing wheals, angioedema, or both, associated with pruritus. Acute forms are frequent and limited to outbreaks of less than 6 weeks; while the chronic ones have a prevalence of less than 1%, longer duration and can be spontaneous or inducible. The etiopathogenic mechanisms involved in this disease include autoallergy, autoimmunity, and inflam mation with cell activation, mainly of the mast cell, leading to its degranulation with the release of vasoac tive mediators. Along its approach, diagnostic confirmation, search for indicators of its etiopathogenesis, detection of cofactors that can modulate its activity, recognition of comorbidi ties, evaluation of possible biomarkers and the assess ment of disease activity, impact and control are essential. The pharmacological management aims to control the symptoms, until the urticaria, which is self-resolv ing, is gone. This is described in a stepwise fashion with increasing complexity.

Psychosocial Burden of Psoriasis: A Systematic Literature Review of Depression Among Patients with Psoriasis.

Psoriasis is associated with various comorbidities with a notable psychosocial burden. This systematic literature review explores the burden of depression in patients with psoriasis, comparing it with that experienced by patients with other chronic medical conditions. Embase via Ovid, PubMed, and Cochrane Database of Systematic Reviews via Ovid were searched for peer-reviewed studies published in English between January 1, 2016 and December 6, 2021 that reported real-world evidence or observational studies involving at least 100 adults (age ≥ 18 years) with general (unspecified) or plaque psoriasis experiencing symptoms of depression (but not restricted to patients with a clinical diagnosis). Any report of depression or suicidality was eligible for inclusion. Systematic literature reviews reporting depression/suicidality in other chronic medical conditions were also included. Statistical analysis was not performed; the study was descriptive only. A total of 1744 records were identified, and after several defined screenings by two independent reviewers for publication year, relevance, and sample size, 82 publications were included. Psoriasis was significantly associated with depression. The prevalence of depression in patients with psoriasis ranged from 0.2% to 74.6%, with incidence from 4.83 to 91.9 per 1000 person-years. The prevalence of depression was generally higher among patients with more severe psoriasis than those with less severe disease (as determined by Psoriasis Area Severity Index [PASI] scoring system) and was more prevalent among women than men with psoriasis. Depression in psoriasis significantly reduced quality of life, including factors such as sexual dysfunction, sleep difficulties, subjective well-being, and addictions. Comorbid hypertension, hyperlipidemia, psoriatic arthritis, obesity, inflammatory bowel disease, diabetes, and statin use were all associated with increased depression risk in patients with psoriasis. This systematic literature review found that the burden of depression in psoriasis is no lower than in other chronic medical conditions. Greater awareness of the psychological impact of psoriasis would improve care and management, which should incorporate psychological interventions.

[Practical update for the diagnosis and treatment of chronic urticaria]. / Actualización práctica de diagnóstico y tratamiento de la urticaria crónica.

Urticaria is a distinctive pattern of inflammatory response of the skin and/or mucous membranes characterized by the sudden appearance of vanishing wheals, angioedema, or both, associated with pruritus. Acute forms are frequent and limited to outbreaks of less than 6 weeks; while the chronic ones have a prevalence of less than 1%, longer duration and can be spontaneous or inducible. The etiopathogenic mechanisms involved in this disease include autoallergy, autoimmunity, and inflammation with cell activation, mainly of the mast cell, leading to its degranulation with the release of vasoactive mediators. Along its approach, diagnostic confirmation, search for indicators of its etiopathogenesis, detection of cofactors that can modulate its activity, recognition of comorbidities, evaluation of possible biomarkers and the assessment of disease activity, impact and control are essential. The pharmacological management aims to control the symptoms, until the urticaria, which is self-resolving, is gone. This is described in a stepwise fashion with increasing complexity.

La urticaria es un patrón distintivo de respuesta inflamatoria de piel y/o mucosas caracterizada por la aparición súbita de ronchas evanescentes, angioedema o ambos, asociados a prurito. Las formas agudas son frecuentes y se limitan a brotes de menos de 6 semanas; mientras que las crónicas tienen una prevalencia menor al 1%, mayor duración y pueden ser espontáneas o inducibles. Los mecanismos etiopatogénicos involucrados en esta enfermedad incluyen la autoalergia, la autoinmunidad y la inflamación con la activación celular, principalmente del mastocito, lo que lleva a su degranulación con liberación de mediadores vasoactivos. En su abordaje son fundamentales la confirmación diagnóstica; la búsqueda de indicadores de su etiopatogenia; la detección de cofactores que pueden modular su actividad; el reconocimiento de comorbilidades; la evaluación de posibles biomarcadores y, el impacto en la calidad de vida, el registro de la actividad y el control de la enfermedad. El manejo farmacológico tiene por objetivo controlar los síntomas, mientras la urticaria resuelve de forma espontánea. Este se describe de forma escalonada con una complejidad creciente.

[Management of ulcers on a segmental hemangioma: A combination of systemic and topical therapies in two pediatric cases]. / Manejo de hemangioma ulcerado segmentario: combinación de tratamiento sistémico y tópico en dos casos pediátricos.

Infantile hemangiomas (IHs) are the most common benign tumors of childhood, and segmental ones are rare and associated with a poor prognosis. While these tumors look harmless, one of their main related complications is ulceration during tumor growth. The management in these cases is extremely challenging, requiring a combination of multiple approaches, some specifically aimed at preventing the proliferation of the hemangioma and others aimed at wound care, pain management, and prevention of further infection. Here we discuss two cases to narrate our experience on the management of this condition and its outcome.

Los hemangiomas infantiles (HI) son los tumores benignos más frecuentes de la infancia; la variante segmentaria es rara y se asocia con un mal pronóstico. Una de sus principales complicaciones es la ulceración durante la fase de crecimiento del tumor, a pesar de no presentar características macroscópicas compatibles con una lesión agresiva. El manejo en estos casos es dificultoso e impone la necesidad de asociar múltiples estrategias, algunas orientadas específicamente a impedir la proliferación del hemangioma y otras orientadas a la curación de la herida, el manejo del dolor y la prevención de la infección agregada. Presentamos dos casos a fin de comunicar nuestra experiencia respecto del manejo de dicha patología y su evolución final.

Manejo de hemangioma ulcerado segmentario: combinación de tratamiento sistémico y tópico en dos casos pediátricos / Management of ulcers on a segmental hemangioma: A combination of systemic and topical therapies in two pediatric cases

Los hemangiomas infantiles (HI) son los tumores benignos más frecuentes de la infancia; la variante segmentaria es rara y se asocia con un mal pronóstico. Una de sus principales complicaciones es la ulceración durante la fase de crecimiento del tumor, a pesar de no presentar características macroscópicas compatibles con una lesión agresiva. El manejo en estos casos es dificultoso e impone la necesidad de asociar múltiples estrategias, algunas orientadas específicamente a impedir la proliferación del hemangioma y otras orientadas a la curación de la herida, el manejo del dolor y la prevención de la infección agregada. Presentamos dos casos a fin de comunicar nuestra experiencia respecto del manejo de dicha patología y su evolución final.

Infantile hemangiomas (IHs) are the most common benign tumors of childhood, and segmental ones are rare and associated with a poor prognosis. While these tumors look harmless, one of their main related complications is ulceration during tumor growth. The management in these cases is extremely challenging, requiring a combination of multiple approaches, some specifically aimed at preventing the proliferation of the hemangioma and others aimed at wound care, pain management, and prevention of further infection. Here we discuss two cases to narrate our experience on the management of this condition and its outcome.

ECHO project in atopic dermatitis in Argentina: An innovative strategy to reach underserved areas with up to date knowledge, first year of experience

Abstract Background: The ECHO® (Extension for Community Healthcare Outcomes) project is a model of distance medical education developed in the United States to support health professionals in the management of patients with complex diseases. Since 2019, it has been implemented in atopic dermatitis (AD) in Argentina. The program consists of the periodic presentation of clinical cases by videoconference, virtual classes, and a permanently available open chat between professionals in charge of patients with AD and a group of experts. Objective: The objective of this study was to analyze the impact of the ECHO Project AD on the medical knowledge and medical skills of Argentinian health professionals when treating patients with AD. Methods: A survey was carried out among the participants in order to evaluate the impact of the program on the care of patients with AD. Results: ECHO Project AD revealed a significant improvement in the management of patients with AD. The program contributed to the interpretation and use of severity scores, use of phototherapy, and management and prescription of both classic and innovative topical and systemic treatments. Study limitations: The reduced number of participants and the short period of time. The answers of the survey may be biased by the enthusiasm of the participants. Conclusions: The ECHO project is an educational tool that enhances the medical skills of doctors and institutions, in which a climate of a partnership comes first and the participants look forward to learning from experiences, successes, and mistakes from one another, producing a scientific hub in constant evolution.

ECHO project in atopic dermatitis in Argentina: An innovative strategy to reach underserved areas with up to date knowledge, first year of experience.

BACKGROUND: The ECHO® (Extension for Community Healthcare Outcomes) project is a model of distance medical education developed in the United States to support health professionals in the management of patients with complex diseases. Since 2019, it has been implemented in atopic dermatitis (AD) in Argentina. The program consists of the periodic presentation of clinical cases by videoconference, virtual classes, and a permanently available open chat between professionals in charge of patients with AD and a group of experts. OBJECTIVE: The objective of this study was to analyze the impact of the ECHO Project AD on the medical knowledge and medical skills of Argentinian health professionals when treating patients with AD. METHODS: A survey was carried out among the participants in order to evaluate the impact of the program on the care of patients with AD. RESULTS: ECHO Project AD revealed a significant improvement in the management of patients with AD. The program contributed to the interpretation and use of severity scores, use of phototherapy, and management and prescription of both classic and innovative topical and systemic treatments. STUDY LIMITATIONS: The reduced number of participants and the short period of time. The answers of the survey may be biased by the enthusiasm of the participants. CONCLUSIONS: The ECHO project is an educational tool that enhances the medical skills of doctors and institutions, in which a climate of a partnership comes first and the participants look forward to learning from experiences, successes, and mistakes from one another, producing a scientific hub in constant evolution.

Atopic Dermatitis in Latin America: A Roadmap to Address Data Collection, Knowledge Gaps, and Challenges.

BACKGROUND: Atopic dermatitis (AD) is a systemic, multifactorial disease that causes significant morbidity and health care burden in Latin America (LA). Data on AD are scarce in LA. Lack of disease registries and non-standardized study methodologies, coupled with region-specific genetic, immunological, and environmental factors, hamper data collection. A panel of LA experts in AD was given a series of relevant questions to address before a conference. Each narrative was discussed and edited through numerous rounds of deliberation until achieving consensus. Identified knowledge gaps in AD research were updated prevalence, adult-disease epidemiology, local phenotypes and endotypes, severe-disease prevalence, specialist distribution, and AD public health policy. Underlying reasons for these gaps include limited funding for AD research, from epidemiology and public policy to clinical and translational studies. Regional heterogeneity requires that complex interactions between race, ethnicity, and environmental factors be further studied. Informed awareness, education, and decision making should be encouraged.

An Argentinean cohort of patients with rheumatic and immune-mediated diseases vaccinated for SARS-CoV-2: the SAR-CoVAC Registry-protocol and preliminary data.

BACKGROUND/OBJECTIVE: To evaluate the efficacy and safety of SARS-CoV-2 vaccine in patients with rheumatic and immune-mediated inflammatory diseases (IMIDs) in Argentina: the SAR-CoVAC registry. METHODS: SAR-CoVAC is a national, multicenter, and observational registry. Adult patients with rheumatic or IMIDs vaccinated for SARS-CoV-2 were consecutively included between June 1 and September 17, 2021. Sociodemographic data, comorbidities, underlying rheumatic or IMIDs, treatments received, their modification prior to vaccination, and history of SARS-CoV-2 infection were recorded. In addition, date and place of vaccination, type of vaccine applied, scheme, adverse events (AE), disease flares, and new immune-mediated manifestations related to the vaccine were analyzed. RESULTS: A total of 1234 patients were included, 79% were female, with a mean age of 57.8 (SD 14.1) years. The most frequent diseases were rheumatoid arthritis (41.2%), osteoarthritis (14.5%), psoriasis (12.7%), and spondyloarthritis (12.3%). Most of them were in remission (28.5%) or low disease activity (41.4%). At the time of vaccination, 21% were receiving glucocorticoid treatment, 35.7% methotrexate, 29.7% biological (b) disease modifying anti-rheumatic drugs (DMARD), and 5.4% JAK inhibitors. In total, 16.9% had SARS-CoV-2 infection before the first vaccine dose. Most patients (51.1%) received Gam-COVID-Vac as the first vaccine dose, followed by ChAdOx1 nCoV-19 (32.8%) and BBIBP-CorV (14.5%). Half of them (48.8%) were fully vaccinated with 2 doses; 12.5% received combined schemes, being the most frequent Gam-COVID-Vac/mRAN-1273. The median time between doses was 51 days (IQR 53). After the first dose, 25.9% of the patients reported at least one AE and 15.9% after the second, being flu-like syndrome and local hypersensitivity the most frequent manifestations. There was one case of anaphylaxis. Regarding efficacy, 63 events of SARS-CoV-2 infection were reported after vaccination, 19% occurred during the first 14 days post-vaccination, 57.1% after the first dose, and 23.8% after the second. Most cases (85.9%) were asymptomatic or mild and 2 died due to COVID-19. CONCLUSIONS: In this national cohort of patients, the most common vaccines used were Gam-COVID-Vac and ChAdOx1 nCoV-19. A quarter of the patients presented an AE and 5.1% presented SARS-CoV-2 infection after vaccination, in most cases mild. STUDY REGISTRATION: This study has been registered in ClinicalTrials.gov under the number: NCT04845997. Key Points • This study shows real-world data about efficacy and safety of SARS-CoV-2 vaccination in patients with rheumatic and immune-mediated inflammatory diseases. Interestingly, different types of vaccines were used including vector-based, mRNA, and inactivated vaccines, and mixed regimens were enabled. • A quarter of the patients presented an adverse event. The incidence of adverse events was significantly higher in those receiving mRAN-1273 and ChAdOx1 nCoV-19. • In this cohort, 5.1% presented SARS-CoV-2 infection after vaccination, in most cases mild.

Abrocitinib monotherapy in Investigator's Global Assessment nonresponders: improvement in signs and symptoms of atopic dermatitis and quality of life.

BACKGROUND: Abrocitinib, a once-daily, oral Janus kinase 1 selective inhibitor, was shown to be an effective treatment for moderate-to-severe atopic dermatitis in phase 2 b/3 monotherapy trials. METHODS: These analyses included data for Investigator's Global Assessment responder (clear [0] or almost clear [1] with ≥2-grade improvement) and nonresponder patients with moderate-to-severe atopic dermatitis who received abrocitinib (200 mg or 100 mg) or placebo in three abrocitinib monotherapy trials (phase 2 b, NCT02780167; two phase 3, NCT03349060/JADE MONO-1 and NCT03575871/JADE MONO-2). Outcomes measuring skin clearance, itch, and quality of life were evaluated. RESULTS: Both nonresponders (n = 548) and responders (n = 260) treated with abrocitinib had rapid and clinically meaningful improvement in skin clearance, itch, and quality of life compared with placebo. CONCLUSION: Patients with moderate-to-severe atopic dermatitis treated with abrocitinib who did not achieve an Investigator's Global Assessment 0/1 response at week 12 still experienced rapid, clinically meaningful improvements across several other validated measures of efficacy and quality of life. CLINICALTRIALS.GOV: NCT02780167, NCT03349060, NCT03575871.

Psoriasis and pregnancy in the biologic era, a feared scenario. What do we do now?

Psoriasis is a chronic, multifactorial inflammatory disease; its clinical severity can vary widely. Treatment of severe cases during pregnancy is of special interest. To date there is scarce information available and most data comes from other medical specialties that use similar treatments. Immunosuppressors are strongly discouraged during pregnancy and breastfeeding. Amongst biologic agents, anti-TNFα having been the longest on the market has allowed for the most experience. It is known that transplacental transport of these drugs does not occur until gestational week 22, once organogenesis is completed. Within this group certolizumab pegol, seems to be the safest choice, as its molecular structure does not cross the placental barrier. Beyond pregnancy, it is important to take into account these drugs' half-life and passage to breast milk, as well as its impact on neonatal immunization.

WhatsApp: A Real-Time Tool to Reduce the Knowledge Gap and Share the Best Clinical Practices in Psoriasis.

BACKGROUND: Psoriasis is a chronic inflammatory disease that affects around 100 million people worldwide. The burden of disease is high, but more recent therapies show promising results. Clinicians need, however, more training in the use of such therapies. INTRODUCTION: Project ECHO® (Extension for Community Healthcare Outcomes) is structured around the promise of delivering medical education at a distance, empowering clinicians who operate in remote areas. The use of instant messaging services, such as WhatsApp® Messenger, has the potential to improve on the existing framework and bridge the existing gap of knowledge. This article reports on a study concerning the implementation of a WhatsApp discussion group in Project ECHO Psoriasis in Argentina. MATERIALS AND METHODS: One hundred thirty-two dermatologists in Argentina were invited to participate in the WhatsApp discussion group. After 1 year of participation, a follow-up questionnaire was used to assess the effectiveness of the project. RESULTS: Eighty dermatologists participated. All questions placed in the discussion were answered by a psoriasis specialist, 79% of which were answered within the first 5 min. Clinicians report significant improvement in diagnosis, comorbidities, and treatment with both conventional and biological therapies. DISCUSSION: Preliminary results are promising. This new cost-effective solution builds on the existing Project ECHO Psoriasis in Argentina and shows potential in bridging the gap of knowledge, promoting better clinical decisions through empowerment of medical doctors operating in remote locations. CONCLUSIONS: Further research is needed to increase generalization of the results. Moreover, it would be interesting to match the data from the discussion group with follow-up questionnaires.

Manifestaciones mucocutáneas de intoxicación por metotrexato / 4pp Mucocutaneous manifestations of methotrexate toxicity

El metotrexato es un antimetabolito análogo al ácido fólico que inhibe competitivamente la enzima dihidrofolato reductasa y timidilato sintetasa, indispensables para la síntesis de ADN y ARN. Se utiliza ampliamente en dermatología y sus efectos adversos en la piel y mucosas son variados, incluyendo reacciones leves y graves. Las erosiones y úlceras cutáneas como manifestación de citotoxicidad por metotrexato son infrecuentes y representarían un signo cutáneo temprano de pancitopenia por toxicidad medular secundaria a dicha droga. En la mayoría de los casos existen enfermedades cutáneas previas a la ulceración, principalmente psoriasis. En ausencia de dermatitis subyacente, la presencia de ulceraciones es excepcional. Se presentan ocho casos de pacientes con signos cutáneos de intoxicación por metotrexato, con y sin dermatosis previas. En la mayoría hubo asociación de mucositis y compromiso medular. Se recomiendan pautas de tratamiento.

Methotrexate is an antimetabolite analog to folic acid that competitively inhibits the enzyme dihydrofolate reductase and thymidylate synthetase, essential for the synthesis of DNA and RNA. It is widely used in dermatology and its adverse effects on the skin and mucous membranes are varied, including mild and severe reactions. The appearance of erosions and skin ulcers as a manifestation of methotrexate cytotoxicity are quite infrequent. These would represent an early cutaneous sign of pancytopenia due to marrow toxicity secondary to this drug. In most of the cases there are cutaneous diseases prior to ulceration, mainly psoriasis. In the absence of underlying dermatitis, the presence of ulcerations is very rare. We present eight cases of patients with cutaneous signs of methotrexate poisoning, with and without previous dermatoses. Most of them associated mucositis and bone marrow involvement. Treatment guidelines are recommended.

[4pp Mucocutaneous manifestations of methotrexate toxicity]. / Manifestaciones mucocutáneas de intoxicación por metotrexato.

Methotrexate is an antimetabolite analog to folic acid that competitively inhibits the enzyme dihydrofolate reductase and thymidylate synthetase, essential for the synthesis of DNA and RNA. It is widely used in dermatology and its adverse effects on the skin and mucous membranes are varied, including mild and severe reactions. The appearance of erosions and skin ulcers as a manifestation of methotrexate cytotoxicity are quite infrequent. These would represent an early cutaneous sign of pancytopenia due to marrow toxicity secondary to this drug. In most of the cases there are cutaneous diseases prior to ulceration, mainly psoriasis. In the absence of underlying dermatitis, the presence of ulcerations is very rare. We present eight cases of patients with cutaneous signs of methotrexate poisoning, with and without previous dermatoses. Most of them associated mucositis and bone marrow involvement. Treatment guidelines are recommended.

Papular Epidermal Nevus with Skyline Basal Cell Layer (PENS): Three New Cases and Review of the Literature.

BACKGROUND/OBJECTIVE: Papular epidermal nevus with skyline basal cell layer (PENS) is a recently described type of epidermal nevus with characteristic histopathologic findings, mainly regular, rectangular acanthosis and a well-demarcated basal cell layer with clear palisading and separation between basal cell nuclei and the first row of Malpighian cell nuclei. Although the first reports described randomly distributed lesions appearing sporadically in otherwise healthy patients, cases of Blaschkoid distribution, lesions associated with extracutaneous manifestations, and familial cases have been reported. METHODS: We performed a review of the clinical charts of all patients with histologic diagnosis of PENS in our hospital. We evaluated epidemiologic, clinical, and histologic features. We then reviewed the literature with a particular emphasis on the presence or absence of extra-cutaneous associations. RESULTS: Three patients with PENS are described. One had a single lesion, one had three lesions, and one, a patient with mild developmental delay, a curved penis, and hypospadias, had multiple lesions. CONCLUSION: The probability of having extracutaneous manifestations is 6.3 times as great in individuals with more than four lesions. Therefore these patients may need closer follow-up.

Dermatologic Aspects of Fabry Disease

Abstract Isolated angiokeratomas (AKs) are common cutaneous lesions, generally deemed unworthy of further investigation. In contrast, diffuse AKs should alert the physician to a possible diagnosis of Fabry disease (FD). Angiokeratomas often do not appear until adolescence or young adulthood. The number of lesions and the extension over the body increase progressively with time, so that generalization and mucosal involvement are frequent. Although rare, FD remains an important diagnosis to consider in patients with AKs, with or without familial history. Dermatologists must have a high index of suspicion, especially when skin features are associated with other earlier symptoms such as acroparesthesia, hypohidrosis, or heat intolerance. Once the diagnosis is established, prompt screening of family members should be performed. In all cases, a multidisciplinary team is necessary for the long-term follow-up and treatment.

Aplasia Cutis Congenita Associated with Fetus Papyraceus.

Aplasia cutis congenita (ACC), or congenital absence of skin, is a heterogeneous condition that may be due to varied mechanisms. ACC has rarely been reported in association with fetus papyraceus, representing a peculiar phenotype of ACC. We present six new cases of neonates born with symmetric ACC associated with intrauterine fetal demise of co-twins during the late first or early second trimester.