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Pustular psoriasis is a serious life-threatening disease, and patients usually show poor response to traditional treatments. In recent years, interleukin-17 and interleukin-23 inhibitors have shown favorable efficacy in the treatment of psoriasis. This review summarizes the latest progress in interleukin-17 and interleukin-23 inhibitors for the treatment of pustular psoriasis.
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Objective:To evaluate clinical efficacy and safety of secukinumab in the treatment of patients with localized pustular psoriasis irresponsive or intolerant to conventional treatment.Methods:Clinical data were collected from 13 patients with refractory localized pustular psoriasis, who received secukinumab treatment in Department of Dermatology, Xijing Hospital from December 2019 to April 2022. Efficacy was evaluated by comparing palmoplantar pustulosis (PPP) area and severity index (PPPASI) score and physician global assessment (PGA) score in PPP patients before and after the treatment, and by comparing clinical global impression (CGI) score in patients with acrodermatitis continua of Hallopeau (ACH). Adverse events were recorded during the treatment.Results:Among the 13 patients with refractory localized pustular psoriasis, 6 were diagnosed with PPP, 3 with ACH, and 4 with PPP complicated by ACH. There were 3 males and 10 females, and their age was 33.2 ± 14.6 years. After 12-week treatment, the PPPASI score decreased from 13.88 ± 3.62 points at baseline to 6.81 ± 2.31 points in 10 patients presenting with PPP lesions, 4 achieved 75% improvement in the PPPASI score (PPPASI75), and 5 achieved PGA0/1; at the same time, 6 of the 7 patients presenting with ACH lesions achieved moderate or marked improvement in the CGI score, and 4 of the 6 patients achieved marked improvement. Two patients with PPP discontinued the treatment after 3- and 5-week treatment respectively due to poor response, and 1 patient with ACH achieved mild improvement in the CGI score after 12-week treatment. No severe adverse events were reported during the treatment. However, inflammatory follicular papules occurred in 1 patient, and eczematoid lesions occurred in another 1 patient, which both regressed after symptomatic treatment.Conclusion:Secukinumab was effective and safe in the treatment of refractory localized pustular psoriasis, and may serve as a new treatment option for refractory PPP and ACH.
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ABSTRACT BACKGROUND: The number of bariatric surgeries performed worldwide is growing. Among the main short, medium or long-term complications after surgery are nutritional deficiencies. Many of these, such as those of Zn, Cu and vitamins A, B1, B3, B6 and B12, are manifested by dermatological lesions before potentially fatal systemic disorders occur. OBJECTIVE: To identify the main dermatological manifestations associated with nutritional deficiencies after bariatric surgery, and the associated variables. DESIGN AND SETTING: Integrative literature review carried out at a public university in Brazil. METHODS: This was a case report and a review of health research portals and databases of national and international biomedical journals, without publication date limitation. The descriptors used for searches followed the ideal methodology for each database/search portal: "bariatric surgery", "skin", "skin disease", "skin manifestation", "deficiency disease" and "malnutrition". RESULTS: A total of 59 articles were selected, among which 23 were review articles or articles that addressed specific dermatological manifestations. The other 36 articles described 41 cases, which were organized into a table with the clinical variables. CONCLUSIONS: Although nutritional deficiencies are expected as complications after bariatric surgery, few articles relating them to their dermatological manifestations were found. It is important to recognize skin changes caused by nutritional deficiencies in patients treated via bariatric surgery, as these may occur before systemic complications appear and are easier to diagnose when the patient does not have any systemic symptoms yet. However, there is generally a delay between the appearance of skin lesions and making the diagnosis of nutritional deficiency.
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Abstract Generalized pustular psoriasis (von Zumbusch) is a rare and acute eruption characterized by multiple sterile pustules over an erythematous and edematous background, eventually associated with psoriasis vulgaris. Classically, it manifests as a potentially severe systemic picture and demands prompt diagnosis and intervention. The duration of each flare-up and intervals between the pustular episodes is extremely variable. Recently, genetic abnormalities have been identified mainly in the familial and early variants of this disease. The therapeutic arsenal is limited; however, new drugs being evaluated aim to control both pustular flare-ups and disease recurrences.
Subject(s)
Humans , Psoriasis/diagnosis , Psoriasis/drug therapy , Skin Diseases, Vesiculobullous , Exanthema , Acute Disease , Chronic DiseaseABSTRACT
RESUMEN Fundamento: el síndrome de Gianotti-Crosti también llamado acrodermatitis papular de la infancia es un exantema infrecuente, que se presenta por lo general en niños entre uno y seis años de edad. Dado que estos pacientes son llevados a su pediatra, es fundamental el conocimiento de las características de este síndrome. Objetivo: conocer un paciente con diagnóstico de síndrome de Gianotti-Crosti, también llamado acrodermatitis papular de la infancia. Presentación del caso: paciente de 18 meses que presentó un exantema pápulo-vesicular asintomática de distribución simétrica en cara, nalgas y superficies extensoras de las extremidades precedido de fiebre. Que duró dos semanas y cedió sin tratamiento. Conclusiones: debe sospecharse la enfermedad en niños con exantema pápulo-vesicular de distribución simétrica en cara, nalgas y superficies extensoras de las extremidades, asintomático y auto limitado.
ABSTRACT Background: the Gianotti-Crosti syndrome, also known as popular acrodermatitis of childhood, is an uncommon rash that usually occurs in children between 1 and 6 years old. Given that these patients are usually brought to their pediatrician, it is essential to know the characteristics of this syndrome. Objective: to show a patient with Gianotti-Crosti syndrome or popular acrodermatitis of childhood. Case report: an 18-month-old female patient, with asymptomatic papulo-vesicular reaction, symmetrically distributed on the face, buttocks and extensor surfaces of the extremities, preceded by fever. It lasted two weeks and disappeared without treatment. Conclusions: the entity should be suspected in children between 1 and 6 years old with asymptomatic papulo-vesicular eruption, symmetrically distributed on the face, buttocks and extensor surfaces of the extremities, asymptomatic and self-limited.
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Resumen Los exantemas virales en la infancia,son motivo deconsulta frecuente, en los Servicios de Dermatología, Pediatría, y Urgencias. Usualmente, son autolimitados y pueden ser difíciles de diagnosticar. Es importante reconocerlos y diferenciarlos de otras enfermedades severas que requieran un tratamiento inmediato. El síndrome de Gianotti-Crosti, también conocido como acrodermatitispapulosa de la infancia, es un exantema poco frecuente, secundario a la respuesta inmune del huésped frente a infecciones, especialmente virales o luego de inmunizaciones. La primera asociación descrita fue con la infección por el virus de hepatitis B, sin embargo, con la introducción de la vacuna contra este virus, aumentó la incidencia de otros virus relacionados con este síndrome. Presentamos el caso de un paciente de 4 años de edad con diagnóstico clínico de síndrome de Gianotti-Crosti.
Abstract Viralexanthemsin childhood are a frequent consultation both outpatient and in emergency services. They are usually self-limited and can be difficult to diagnose. It's important to recognize and differentiate them from other severe diseases that require immediate treatment. GianottiCrosti syndrome, also known as papularacrodermatitis of childhood, is an infrequent exanthema secondary to the host's immune response to infections, especially viral, or vaccines. The first association described was with hepatitis B virus infection, however, with the introduction of the vaccine against this virus,the incidence of other viruses related to this syndrome has increased. We present the case of a 4-year-old patient with a clinical diagnosis of GianottiCrosti syndrome.
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Introducción: La acrodermatitis enteropática es una dermatosis nutricional heredada o adquirida por deficiencia de zinc que clínicamente se caracteriza por eritema, escamocostras y erosiones, especialmente en la región perioral, en zonas acras y en la región anogenital. Además de una ingesta oral inadecuada, hay causas secundarias de esta deficiencia nutricional. Objetivo: Referir una dermatosis nutricional secundaria infrecuente en lactante femenina. Presentación de caso: Pequeñita de 6 meses de edad, fototipo III, alimentada con lactancia materna exclusiva, que acude a consulta especializada de dermatología en el hospital William Soler por cuadro clínico de 3 meses de evolución caracterizado por lesiones eritematocostrosas en regiónes acrales y periorificiales. Los padres refirieron lactancia materna exclusiva. Se realizaron complementarios Conclusiones: Las deficiencias de vitaminas y oligoelementos pueden ocasionar un amplio rango de hallazgos mucocutaneos. En el caso presentado diagnosticado como acrodermatitis enteropática adquirida, hay un aporte inadecuado de zinc en la leche materna(AU)
Introduction: Acrodermatitis enteropathica is an inherited or acquired nutritional dermatosis by zinc deficiency that is clinically characterized by erythema, squamous crusts and erosions, especially in the perioral region, in acral areas and in the anogenital region. In addition to inadequate oral intake, there are secondary causes of this nutritional deficiency. Objective: To refer a nutritional dermatosis uncommon in female infants. Case presentation: 6-months-old baby girl, skin phototype III, fed with exclusive breastfeeding whom was attended in the specialized consultation in Dermatology of William Soler Pediatric Hospital due to clinical framework of 3 months of evolution characterized by eritemato crusty lesions in acral and periorifices´ regions. Parents commented on exclusive breastfeeding. There were made complementary blood tests. Conclusions: The deficiencies of vitamins and trace elements may cause a wide range of mucocutaneous findings. In the presented case diagnosed as adquired acrodermatitis enteropathica, there is an inadequate intake of zinc in breast milk(AU)
Subject(s)
Humans , Female , Infant , Acrodermatitis/diagnosis , Acrodermatitis/epidemiologyABSTRACT
La acrodermatitis enteropática (AE) es una genodermatosis autosómica recesiva causada por la mutación del gen responsable de codificar a la proteína transportadora de Zinc (Zn) SLC39A4. A pesar de ser una rara enfermedad es de fácil manejo y gran relevancia clínica. Se caracteriza por la siguiente tríada: dermatitis acral y periorificial, diarrea y alopecia. Comunicamos un caso de presentación atípica en una lactante de 6 meses de edad con lesiones periorificiales y ampollas acrales que resolvió rápidamente con la terapia suplementaria con Zinc (AU)
Enteropathic acrodermatitis is an autosomal recessive genodermatosis caused by the mutation of the gene responsible for encoding the Zinc transporter protein SLC39A4. Despite being a rare disease, it is easy to manage and of great clinical relevance. It is characterized by the following triad: acral and periorificial dermatitis, diarrhea and alopecia. We report a case of atypical presentation in an almost 6-month-old infant with periorificial lesions and acral blisters that quickly resolved with supplemental Zinc therapy (AU)
Subject(s)
Humans , Female , Infant , Zinc/deficiency , Acrodermatitis/diagnosis , Gastrointestinal Diseases/diagnosis , Acrodermatitis/drug therapy , Zinc Sulfate/therapeutic use , Gastrointestinal Diseases/drug therapyABSTRACT
La paraqueratosis pustulosa es una entidad poco descrita en la literatura y se define como un proceso inflamatorio cutáneo, ungueal y periungueal en el área distal de un dedo habitualmente pulgar o índice, frecuentemente en la infancia. Su evolución suele ser benigna y la respuesta a emolientes tópicos es favorable. A continuación, se presenta un caso de esta enfermedad con el objetivo de resaltar su consideración en patologías ungueales pediátricas.
Pustular parakeratosis is an entity scantly described in literature. It has been described as a skin, nail, and periungual inflammatory process in the distal area of a finger, usually the thumb or index finger, frequently in childhood. Its evolution is usually benign and management is favorable with topical emollients. A clinical case is presented, to raise awareness of this entity in pediatric nail pathologies.
Subject(s)
Humans , Male , Child , Parakeratosis/diagnosis , Parakeratosis/drug therapy , Nails/pathologyABSTRACT
Un hombre de 44 años, previamente sano, consultó por poliartralgias asimétricas de características inflamatorias de tres meses de evolución, que comprometió pequeñas y grandes articulaciones. Poco tiempo después desarrolló pústulas periungueales sobre una base eritematosa y degeneración progresiva de la lámina ungueal del pulgar izquierdo, limitando severamente su funcionalidad. Destacó al examen físico la presencia de placas eritemato-escamosas y pustulosas bien delimitadas en el primer y tercer dedo de la mano izquierda con onicodistrofia severa, que fue un aspecto clave para establecer el diagnóstico de acrodermatitis continua de Hallopeau. Adicionalmente, se observaron otros hallazgos del espectro psoriático: parches eritematosos en el escroto, placas eritemato-escamosas con costras hemorrágicas en ambas rodillas y dactilitis. Se confirmó histológicamente el diagnóstico de psoriasis inversa, psoriasis en placas y psoriasis pustular, respectivamente y con los criterios de Classification Criteria for Psoriatic Arthritis, CASPAR, el diagnóstico de artritis psoriática.
A 44-year-old man, previously healthy, consulted for a three-month history of asymmetrical polyarthralgia with inflammatory features involving small and large joints. A few days later, he developed erythema covered by pustules in the nail folds and progressive degeneration of the nail plate of the left thumb, with severe functional limitation. The physical exam showed well-defined erythematous scaly and pustular plaques in the first and third fingers of the left hand, with severe onychodystrophy, which was a key aspect in the diagnosis of acrodermatitis continua of Hallopeau. Other signs of the psoriatic spectrum were observed: erythematous patches of the scrotum, erythematous scaly plaques with hemorrhagic crusts on both knees, and dactylitis. The diagnosis of inverse psoriasis, plaque psoriasis, and pustular psoriasis, respectively, were confirmed by histopathology and, with the CASPAR criteria, psoriatic arthritis. This case is of particular dermatological interest due to the variety of psoriatic manifestations recognized in a single patient and because of the poorly described association between acrodermatitis continua of Hallopeau with psoriatic arthritis.
Subject(s)
Humans , Male , Adult , Psoriasis/diagnosis , Acrodermatitis/diagnosis , Arthritis, Psoriatic/diagnosis , Psoriasis/pathology , Acrodermatitis/pathology , Arthritis, Psoriatic/pathologyABSTRACT
ABSTRACT Introduction: The treatment of acquired acrodermatitis enteropathica involves zinc supplementation; however, further research is required to establsih the optimal dose and duration of the supplementation. Case presentation: Female patient with a history of gastric bypass, intestinal resection, and 2 months of skin rash that required biopsy with histological findings compatible with dermatitis associated to nutritional deficiency. She received elemental zinc supplementation for 11 days, achieving improvement in skin lesions. She was later readmitted twice due to reactivation of the disease with the need to restart zinc supplementation. At the time of this study, she had been receiving oral elemental zinc treatment for 3 years, at doses of up to 240 mg/day. Discussion: In patients with gastric bypass and intestinal malabsorption, the usual zinc dose of 8-11 mg/day may be insufficient and put patients at risk for acquired acrodermatitis enteropathica. In similar case reports, the supplementation dose ranges from 2 mg/kg/day of venous elemental zinc to 300 mg/day orally, while the duration of supplementation has not been established. Conclusions: Oral supplementation for 3 years with a maximum dose of 240 mg/day has been adequate for controlling the disease. Monitoring blood zinc levels and physical examination of the skin have been key factors for adjusting the dose to be supplied.
RESUMEN Introducción. El tratamiento de la acrodermatitis enteropática adquirida es la suplementación de zinc, sin embargo la dosis óptima y la duración de esta aún son objeto de estudio. Presentación del caso. Paciente femenino con antecedente de bypass gástrico, resección intestinal y 2 meses de erupción cutánea que ameritó biopsia lesional con hallazgos histológicos compatibles con una dermatitis carencial. La mujer recibió suplemento de zinc elemental por 11 días, con lo que se logró mejoría de lesiones cutáneas. Posteriormente, presentó 2 reingresos por reactivación de la enfermedad con necesidad de reiniciar suplementación y, hasta el momento de la presente investigación, había permanecido 3 años con zinc elemental oral con dosis de hasta 240 mg/día. Discusión. En pacientes con malabsorción intestinal la dosis de zinc de rutina de 8-11 mg/ día puede ser insuficiente y los puede colocar en riesgo de acrodermatitis enteropática adquirida. En reportes de caso similares la dosis de suplementación va desde 2 mg/kg/día de zinc elemental venoso hasta 300 mg/día oral, en tanto la duración de la suplementación no ha sido establecida. Conclusiones. La suplementación oral por 3 años con hasta 240 mg/día ha sido adecuada para el control de la enfermedad; el seguimiento a través de la medición de los niveles de zinc en sangre y la exploración física de la piel ha sido clave en el ajuste de la dosis a suplementar.
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Resumen Introducción: La escabiasis costrosa (EC) es una variante poco común de sarcoptiosis clásica, altamente contagiosa. Las lesiones poseen una elevada concentración del ácaro Sarcoptes scabiei var hominis, lo que conlleva a un cuadro clínico más extenso que en la escabiasis clásica. Se observa principalmente en pacientes con algún tipo de inmunocompromiso y se relaciona con el síndrome de Down. Caso clínico: Se describe una paciente pediátrica con síndrome de Down quien presentó placas escamosas que afectaron la porción distal de los dedos, asociadas con distrofia ungueal e hiperqueratosis subungueal, por lo que se consideró acrodermatitis continua de Hallopeau como diagnóstico diferencial. Se realizó una biopsia tipo punch con lo que se llegó al diagnóstico de EC. La paciente recibió tratamiento sistémico con ivermectina vía oral y tratamiento tópico con crema hidratante y desonida al 0.1%. Mostró mejoría clínica notoria dos semanas después de finalizar el tratamiento. Conclusiones: La EC es una variante prevalente en pacientes inmunocomprometidos y con síndrome de Down que fácilmente puede confundirse con patologías inflamatorias con alteración de la queratinización epidérmica. Este caso se considera una presentación atípica debido a la afección localizada en los dedos de las manos asociada con distrofia ungueal. El estudio histopatológico fue necesario para realizar el diagnóstico y descartar diagnósticos diferenciales.
Abstract Background: Crusted scabies (CS) is an uncommon, highly contagious, variant of classic scabies. Elevated concentrations of the mite Sarcoptes scabiei var. hominis are found in the skin lesions, which lead to a more exaggerated clinical picture than in classic scabies. This disease is mainly observed in patients with any kind of immunosuppression and relates to Down syndrome. Case report: A pediatric female patient with Down syndrome, who presented a crusty white plaque associated with nail dystrophy and subungual hyperkeratosis affecting the distal portion of the fingers is described. Because of these findings, the diagnosis of acrodermatitis continua of Hallopeau was considered. A punch biopsy was performed, attaining the diagnosis of CS. She received systemic treatment with oral ivermectin, topical treatment with emollient cream and desonide 0.1%. Notorious clinical improvement was observed two weeks after finalizing treatment. Conclusions: CS is variant of scabies prevalent in immunocompromised patients and Down syndrome that can be easily confused with inflammatory pathologies with abnormal epidermal keratinization. This case is considered as an atypical presentation of the disease because of local affection of the fingers and nail dystrophy. The histopathological study was necessary to obtain the diagnosis and rule out differential diagnosis.
Subject(s)
Animals , Child , Female , Humans , Scabies/diagnosis , Acrodermatitis/diagnosis , Down Syndrome/complications , Sarcoptes scabiei , Scabies/pathology , Scabies/drug therapy , Acrodermatitis/pathology , Ivermectin/administration & dosage , Desonide/administration & dosage , Diagnosis, Differential , Anti-Inflammatory Agents/administration & dosage , Antiparasitic Agents/administration & dosageABSTRACT
Abstract: We report the case of a male 22-month-old child, with atypical presentation of Gianotti-Crosti syndrome after infection with Epstein-Barr virus.
Subject(s)
Humans , Male , Infant , Acrodermatitis/pathology , Acrodermatitis/virology , Skin Diseases, Vesiculobullous/pathology , Skin Diseases, Vesiculobullous/virology , Epstein-Barr Virus Infections/pathology , Remission, Spontaneous , PhotographyABSTRACT
Resumen: Introducción: La acrodermatitis enteropática es una enfermedad de baja incidencia que ocurre por deficiencia de zinc; puede ser hereditaria o adquirida. Se caracteriza por dermatitis acral, alopecia, diarrea y problemas de crecimiento. La afección dermatológica puede simular una infección micótica cutánea u otras enfermedades de la piel relacionadas con patógenos. Caso clínico: Se informa el caso de una paciente de sexo femenino de 7 meses de edad, que fue enviada al Centro Médico Nacional 20 de Noviembre por sospecha de inmunodeficiencia y micosis cutánea. Inició con un cuadro clínico caracterizado por dermatosis diseminada a cabeza, tronco y región genital, por lo que recibió tratamiento inicial con un antifúngico y antibióticos de amplio espectro, sin presentar mejoría. A su ingreso se descartaron inmunodeficiencia e infección micótica. Se sospechó acrodermatitis enteropática, corroborándose el diagnóstico al encontrar niveles séricos de zinc disminuidos. La paciente presentó mejoría inmediata tras el inicio del tratamiento con zinc por vía oral. Conclusiones: El diagnóstico diferencial de acrodermatitis enteropática es amplio e incluye infecciones cutáneas, por lo que el reconocimiento temprano de las características de las lesiones favorece la sospecha, el diagnóstico y el tratamiento adecuado.
Abstract: Introduction: Acrodermatitis enteropathica is a low-incidence disease due to inherited or acquired zinc deficiency. It is characterized by acral dermatitis, alopecia, diarrhea and growth retardation. The dermatological condition may mimic a cutaneous fungal infection or other pathogen-related skin diseases. Case report: We report the case of a female patient of 7 months of age, who was sent to Centro Médico Nacional 20 de Noviembre for suspicion of immunodeficiency and cutaneous mycosis. Her condition began with dermatosis disseminated to the head, trunk and genital region; initial treatment with antifungal and broad spectrum antibiotics was given, without improvement. Upon admission, immunodeficiency and fungal infection were discarded. Acrodermatitis enteropathica was suspected, and corroborated later by low serum zinc levels. Immediately after the start of oral treatment with zinc, the patient showed improvement. Conclusions: There are multiple differential diagnoses of acrodermatitis enteropathica, which includes cutaneous infections. Therefore, the early recognition of the characteristic lesions favors suspicion, diagnosis and appropriate treatment.
ABSTRACT
Acrodematitis enteropathica is an uncommon disease due to zinc deficiency which is characterized by perioral and acral dermatitis, alopecia, and diarrhea. This disease typically presents in infants, either due to an autosomal recessive genetic disorder or after cessation of breastfeeding. Acquired adult-onset acrodermatitis enteropathica has rarely been reported so far. We report a case of a 38-year-old man who had a history of chronic alcohol intake and presented with severe acrodermatitis enteropathica. The patient was successfully managed with zinc supplement and supportive care.
Subject(s)
Adult , Humans , Infant , Acrodermatitis , Alopecia , Breast Feeding , Dermatitis , Diarrhea , ZincABSTRACT
Acrodermatitis enteropathica (AE) is a rare genetic autosomal recessive disorder characterised byperiorificial inflammatory rash, diarrhoea and hair loss. Hereby, we report 3 cases of acrodermatitisenteropathica.
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Introducción: Las manifestaciones dermatológicas en el momento del diagnóstico de fibrosis quística son infrecuentes. Objetivo: Describir el caso de una lactante con una presentación dermatológica no habitual de fibrosis quística. Caso Clínico: Lactante que a los 2 meses de edad presenta un mal incremento pondoestatural. A los 4 meses presenta exantema cutáneo, edema y pelo gris. Dentro de su estudio se evidencia hipoproteinemia y anemia, y se confirma el diagnóstico de fibrosis quística mediante el estudio genético. Las alteraciones dermatológicas se revirtieron completamente tras la instauración de la terapia de reemplazo con enzimas pancreáticas. Este es el segundo caso reportado de pelo gris en niños con esta enfermedad. Conclusión: En niños con malnutrición que desarrollan alteraciones de la piel y fanéreos debemos sospechar enfermedades metabólicas como fibrosis quística.
Introduction: Cutaneous manifestations at the time of CF diagnosis are rare. Objective: To describe the case of an infant with an unusual cutaneous presentation of cystic fibrosis. Case report: The case is presented of an infant with delayed physical growth at two months, and at the age of four months, presented with a skin rash and gray hair. Tests revealed the presence of hypoproteinemia and anemia, with the diagnosis of Cystic Fibrosis being confirmed by genetic testing. The rash was completely resolved after pancreatic enzyme replacement therapy. This is the second gray hair case reported in children with this disease. Conclusion Metabolic diseases such as cystic fibrosis should be suspected in malnourished children who develop skin disorders.
Subject(s)
Humans , Female , Infant , Zinc/deficiency , Acrodermatitis/etiology , Hair Color , Cystic Fibrosis/diagnosis , Enzyme Replacement Therapy/methods , Genetic Testing/methods , Cystic Fibrosis/complications , Hypoproteinemia/etiology , Anemia/etiologyABSTRACT
Se presenta el caso de un lactante del sexo femenino de nueve meses de vida, con una dermatitis generalizada caracterizada por: eritema, descamación y formaciones escamo-costrosas secas y adherentes de tinte amarillento, zonas atrófico-blanquecinas en área genito-perianal y alopecia, cursando con cuadros diarreicos a repetición. Representó un desafío diagnóstico para los médicos tratantes.
A nine-months old baby girl with a generalized dermatitis characterized by: erythema, desquamation and dry and adherent scale-crusty formations of yellowish tint, white atrophic lesions in the genito-perianal area and alopecia, with diarrheal episodes of repetition. It represented a diagnostic challenge to the physicians.
ABSTRACT
Acrodermatitis enteropathica is an uncommon disease caused by hereditary or acquired zinc deficiency. It is characterized by a triad of alopecia, diarrhea and acraland periorificial dermatitis. It is treated with Zinc supplementation. We report a31-year-old indigent and drug addict female with a cutaneous-mucous syndromecharacteristic of acrodermatitis enteropathica. She had a positive clinical evolutionafter Zinc supplementation.
Subject(s)
Adult , Female , Humans , Acrodermatitis/pathology , Dermis/pathology , Zinc/deficiency , Acrodermatitis/diagnosis , Acrodermatitis/drug therapy , Biopsy , Diagnosis, Differential , Zinc/metabolism , Zinc/therapeutic useABSTRACT
La acrodermatitis continua de Hallopeau (ACH) es una enfermedad inflamatoria crónica que afecta a los dedos de las manos y/o pies, se caracteriza por una placa eritematodescamativa con erupciones pustulosas estériles. Es una patología rara o quizá subdiagnosticada y más frecuente en mujeres de edad mediana, según los pocos casos reportados. Se presenta el siguiente caso clínico de una adolescente de 15 años de edad, que presentó una placa eritematodescamativa con pústulas estériles, en tres oportunidades a lo largo de dos años. El diagnóstico de ACH se realizó por la clínica y la histología. Se indicó tratamiento con acitretina vía oral y se tiene mejoría clínica importante.
The acrodermatitis continua of Hallopeau (ACH) is a chronic inflammatory disease that affects the fingers and toes, is characterized by erythematous scaly plaque with sterile pustular eruptions. It is a rare condition or perhaps underdiagnosed, according the few reported cases is more common in middle aged women. We report the case of a 15 year-old woman which presented an erythematous plaque with sterile pustules on three occasions along two years, the diagnosis of ACH was made by clinical and histology. Acitretin therapy is indicated orally and has significant clinical improvement.