Liquen escleroso atrófico con vitiligo en pediatría / Lichen Sclerosus Atrophicus with Vitiligo in Pediatrics

RESUMEN Fundamento: el liquen escleroso es una dermatosis inflamatoria crónica de la piel y semimucosas. Afecta principalmente la vulva y la región perianal, la localización extragenital vista entre un 15 y un 20 % de los casos es rara en la infancia. Objetivo: describir una serie de 8 pacientes en edad pediátrica con padecimiento de liquen escleroso en el Hospital Pediátrico Universitario Paquito González Cueto de Cienfuegos. Métodos: se realizó un estudio descriptivo, retrospectivo, de serie de casos de 8 pacientes seguidos en consulta externa de dermatología del Hospital Pediátrico Universitario Paquito González Cueto de Cienfuegos. La información se extrajo de las historias clínicas de los pacientes. Se tuvo en cuenta la edad expresada en años, sexo, raza, afectación genital, tiempo de evolución en años, síntomas asociados, otras enfermedades asociadas: vitiligo, dermatomiositis, esclerodermia, lupus eritematoso, tipo de lesiones, morfea, fenómeno de koebner y el tratamiento. Resultados: se describieron 8 casos todos en pacientes pediátricos con edad media de 8 años y de evolución de 3,1 años, predominó el sexo femenino, el 37,5 % de la serie presentaron lesiones extragenitales y vitiligo en el 62,5 %, hubo retraso diagnóstico en un paciente. Conclusiones: el liquen escleroso es una patología que afecta la calidad de vida de los pacientes, su posible repercusión sexual, urinaria y su potencial riesgo de carcinoma epinocelular, por lo que se hace necesario realizar un diagnóstico oportuno de este padecimiento.

ABSTRACT Background: lichen sclerosus is a chronic inflammatory dermatosis of the skin and semi-mucous membranes. It mainly affects the vulva and the perianal region, the extragenital location seen between 15 and 20 % of cases, is rare in childhood. Objective: to describe 8 pediatric patients with lichen sclerosus at the Paquito González Cueto Cienfuegos University Pediatric Hospital. Methods: a descriptive, retrospective study of 8 patients followed in the dermatology outpatient clinic of the Paquito González Cueto University Pediatric Hospital in Cienfuegos was carried out. The information was extracted from the medical records of the patients. Age expressed in years, sex, race, genital involvement, evolution time in years, associated symptoms, other associated diseases were taken into account: vitiligo, dermatomyositis, scleroderma, lupus erythematosus, type of lesions, morphea, koebner and treatment. Results: 8 cases were described, all in pediatric patients with a mean age of 8 years and an evolution of 3.1 years, predominantly female, 37.5 % of the series had extragenital lesions and vitiligo 62.5 %, there were diagnostic delay in a patient. Conclusions: lichen sclerosus is a pathology that affects the quality of life of patients, its possible sexual and urinary repercussions and its potential risk of epinocellular carcinoma, for which it is necessary to make a timely diagnosis.

Morphea: a practical review of its diagnosis, classification and treatment

Morphea, or localized scleroderma, is a rare disease of the connective tissue that manifests itself with localized sclerosis of the skin and, in some cases, with extracutaneous manifestations. Its etiology is not fully understood, but it is believed that there is genetic predisposition, in addition to environmental triggering factors. Classification of the disease is not simple due to its multiple presentations; however, it is useful in order to define the treatment, which should be individualized and started early to avoid cosmetic and functional complications. In this review, we summarize the most important practical aspects of the classification, diagnostic methods and evaluation of morphea activity, as well as available therapeutic options, with an emphasis on existing clinical evidence regarding their efficacy and safety.

Liquen escleroso y atrófico de localización extragenital / Extragenital lichen sclerosus et atrophicus

RESUMEN El liquen escleroso y atrófico (LEA), es una enfermedad muco-cutánea inflamatoria crónica cuya etiología es desconocida. Se lo ha vinculado con diversos factores: inmunológicos, genéticos, hormonales, infecciosos y traumáticos. Su localización habitual es la genital. Las formas extra genitales son poco frecuentes y suelen afectar tronco, cuello y extremidades. Se presenta un caso de liquen escleroso y atrófico de localización extra genital, en una paciente de 14 años de edad.

SUMMARY Lichen sclerosus et atrophic (LSA) is a chronic inflammatory mucocutaneous disease of unknown etiology. It has been linked to various immunological, genetic, hormonal, infectious and traumatic factors. Its usual location is the genital area. Extra-genital forms are infrequent and usually involve trunk, neck, and extremities. We present the case of a 14 years old female with lichen sclerosus et atrophic of extragenital localization.

Extragenital Lichen Sclerosus Et Atrophicus along the Blaschko's Lines of the Forehead / 대한피부과학회지

Lichen sclerosus et atrophicus (LSA) is a chronic inflammatory dermatosis presenting as white papules and atrophic patches in the anogenital region. Extragenital LSA is rare and commonly affects the neck, thighs, and the trunk. A 68-year-old woman presented with a well-demarcated, whitish linear plaque on the forehead that was observed several months prior to presentation. A biopsy specimen showed hyperkeratosis and thinning of the epidermis and homogenization of collagen in the papillary dermis with a subepidermal cleft. Mild periappendageal lymphocytic infiltration and eccrine gland atrophy secondary to thickening of collagen bundles were observed in the deep dermis. Based on the clinical and histopathological findings, this patient was diagnosed with extragenital LSA and concomitant morphea along the Blaschko's lines.

Clinical and reflectance confocal microscopy features of 11 cases of extragenital lichen sclerosus et atrophicus / 中华皮肤科杂志

Objective To investigate clinical and reflectance confocal microscopy(RCM)features of extragenital lichen sclerosus et atrophicus.Methods Clinical data and RCM images were collected from 11 patients with extragenital lichen sclerosus et atrophicus in Department of Dermatology of Shenyang No.7 People's Hospital and the First Hospital of China Medical University between October 2010 and October 2016,and retrospectively analyzed.Results Of the 11 patients,9 were female,and 6 had lesions on the neck.RCM imaging showed atrophy of the epidermis,disappearance of high reflectivity and circular structures in the basal layer,blurred dermo-epidermal junction,highly reflective quasi-circular big cells and a few mononuclear cells scattering in the superficial dermis,and homogenization and increased density of collagen.Conclusion Extragenital lichen sclerosus et atrophicus has characteristic RCM presentation.

Liquen escleroso y atrófico genital o craurosis de la vulva. Presentación de un caso / Sclerosus and atrophic genital Lichen or vulvar craurosis. About a case

El liquen escleroso y atrófico o la craurosis de la vulva es una afección caracterizada por la atrofia progresiva crónica de la piel y la mucosa vulvar, asociada con cambios involutivos en los genitales externos. El que sea muy poco usual en nuestro medio fue la causa que motivó la presentación de este caso, de una paciente de 49 años, de color de piel blanca, con antecedentes patológicos personales de hipertensión arterial, que asistió a consulta de Dermatología remitida por su médico de familia. Refirió que desde hacía más o menos ocho meses padecía de prurito intenso en la región vulvar y cambios en la textura de la misma que señaló como ¨sensación de endurecimiento¨ que se había intensificado, así como irregularidad en el período menstrual, todo lo cual no se resolvía con tratamiento habitual pues siempre fue interpretado como parásitos vaginales. Explicó que sufría de dolor intenso durante las relaciones sexuales, con mayor frecuencia en los últimos meses, así como trastornos del sueño y nerviosismo. Se realizó biopsia que arrojó como diagnosticó un liquen escleroso. Se le puso tratamiento con esteroides locales de alta potencia, estrógenos conjugados y apoyo psicológico. La paciente tuvo una evidente mejoría del cuadro sintomático. Se le realiza evaluación periódica para evitar recaídas y/o complicaciones.

Sclerosus and atrophic genital Lichen or vulvar craurosis is an affection characterized by a progressive chronic atrophy of the vulvar skin and mucosa, associated to involutive changes in external genitalia. The presentation of this case was motivated by the unusual appearance of it in our context. A 49 year old white patient with history of hypertension, came to the Dermatology consultation referred by her Family Doctor. She reported that she had had an intense pruritus since approximately eight months in the vulvar region and changes in texture which she stated as ¨hardening sensation¨ which had had intensified, so as irregularities in her menstrual period, all of which was not relieved with the usual treatment as it was interpreted as vaginal parasites. She explained that she suffered from an intense pain during intercourse, more frequently in the last months so as sleeping problems and nervousness. A biopsy was performed and it showed lichen sclerosus. It was treated with local high potency steroids, conjugated estrogens and psychological support. The patient had an evident symptom improvement. She has a periodic evaluation to avoid relapse and /or complications.

Extensive bullous lichen sclerosus et atrophicus

Abstract Lichen sclerosus et atrophicus is a chronic disease of unknown etiology characterized by atrophic and sclerotic plaques in both genital and extragenital regions. Extensive bullous lichen sclerosus et atrophicus (BLSA) is a severe variant of the disease with no widely accepted treatment. We present a 63-year-old woman with extensive extragenital, ivory-colored, atrophic plaques on her trunk and extremities and disseminated hemorrhagic bullae. The patient was unsuccessfully treated with standard topical corticosteroid therapy, doxycycline and chloroquine. According to the literature, there is little evidence of the efficacy of doxycycline and hydroxychloroquine in the treatment of BLSA. We report a rare case of extensive BLSA that is unresponsive to these drugs.

Lichen sclerosus associated with localized scleroderma: dermoscopy contribution

Abstract: Lichen sclerosus is an uncommon inflammatory dermatosis with preferential involvement of the urogenital region. The extragenital involvement is uncommon and is characterized by small rounded macules or papules, pearly white in color. The coexistence of lichen sclerosus and scleroderma plaques in most cases with extragenital location has been reported in the literature. We report a case of lichen sclerosus associated with scleroderma in children, highlighting the importance of dermoscopy in diagnosis.

Dermatoscopic characteristics of 15 cases of lichen sclerosis / 中华皮肤科杂志

Objective To assess dermatoscopic characteristics of lichen sclerosus(LS). Methods Dermatoscopy was performed to observe 27 genital or extragenital porcelain-white skin lesions in 15 outpatients with confirmed LS. Results As dermatoscopy showed, of the 27 skin lesions, 24 showed white-yellowish structureless areas, 25 linear vessels, 27 white patches, 17 keratotic plugs. Characteristic cloverleaf-like structure was observed in 7 skin lesions in 4 patients, which was consistent with LS. Conclusion Patients with LS show atypical clinical manifestations, but specific dermatoscopic patterns.

Liquen escleroso extragenital: Presentación de un caso / Lichen scleros extragenital: presentation of a case

El liquen escleroso es una enfermedad crónica inflamatoria de causa desconocida. En hombres su presentación es infrecuente y se observa más comúnmente en la cuarta década de la vida. Asimismo, las lesiones extragenitales son inusuales y asientan más comúnmente en extremidades y tronco. A continuación, presentamos un paciente de sexo masculino adolescente con diagnóstico de liquen escleroso en dorso

Lichen sclerosus is a chronic inflammatory disease of unknown etiology. Appearance in men is unusual and is most seen at fourth decade of life. Also extragenital lesions are rare and most commonly settle in extremities and trunk. A case of a male adolescent patient with lichen sclerosus in trunk is reported.

Balanitis xerótica obliterante en el adulto: A propósito de un caso / Balanitis xerotica obliterans: A case report in an adult

El liquen escleroso y atrófico (LE y A) del pene, también conocido como balanitis xerótica obliterante (BXO), es una enfermedad inflamatoria crónica del pene que puede ocurrir a cualquier edad. La inflamación conduce a la formación de placas hipocrómicas, más comúnmente en el prepucio o en el glande. En general puede causar fimosis, prurito, dolor por las fisuras, disuria, restricción de la micción, dispareunia y disfunción sexual significativa. Comunicamos un caso de BXO asociado a vitiligo en un paciente de 78 años, quien consultó por dispareunia. Fue derivado a Urología para resolución quirúrgica, donde se realizó una postectomía, cuyo estudio era compatible con una BXO. Muchos hallazgos obtenidos en los últimos años apuntan cada vez más, hacia un origen autoinmune de esta enfermedad. El uso de corticoides tópicos de alta potencia es todavía la mejor terapéutica, siendo la cirugía un buen tratamiento en casos muy severos, con fimosis importante o aquellos que no responden al uso de corticoides o inhibidores de la calcineurina, como el tacrolimus o pimecrolimus.

Lichen sclerosus et atrophicus (ALE) of the penis, also known as balanitis xerotica obliterans (BXO) is a chronic inflammatory disease of the penis that can occur at any age. The inflammation leads to the formation of hypochromic plaques, most commonly in the foreskin or on the glans. BXO can cause phimosis, pruritus, pain, dysuria, urinary restriction, dyspareunia, and significant sexual dysfunction. We present a case of BXO associated to vitiligo in a 78 years old patient, who consulted for dyspareunia. It was referred to urologist for surgical resolution, where circumcision was performed with biopsy compatible with BXO. In recent years studies have found that it could exists, an autoimmune ethiopathogenic factor of the disease. The topical use of high power corticosteroids is still the first choice and surgery remains a good treatment in severe cases (ex. phimosis) or those who do not respond to steroids.

Bullous and hemorrhagic lichen sclerosus - Case report

Lichen sclerosus is a chronic inflammatory disease, usually located in the genital area. The etiology of lichen sclerosus is multifactorial, with participation of genetic, autoimmune, infectious and hormonal factors. Bullous clinical form stems from hydropic degeneration of the basal membrane, constituting a less frequent variant of the disease. In this work, we report the case of a female patient, 55 years old, who in the last three years presented whitish plaques, with horny spikes, located on back and arms. Some of these lesions evolved with hemorrhagic blisters, which after histopathological examination confirmed the diagnosis of bullous and hemorrhagic lichen sclerosus. The patient was treated with high-potency topical corticosteroid for two months, resulting in remission of bullous and hemorrhagic lesions.

Perineostomy: the last oportunity

Objective To review the technique and outcome of perineal urethrostomy or urethral perineostomy and to identify factors related to the procedure failure. Material and methods We studied 17 patients who underwent perineal urethrostomy between 2009-2013 in a single hospital. Success was defined as no need for additional surgical treatment or urethral dilatation. We reviewed the clinical data related to age, weight, previous urethral surgery, diabetes, hypertension, ischemic cardiopathy, lichen sclerosus and other causes and studied their association with the procedure failure (univariate analysis). We completed the analysis with a multivariate test based on binary regression. Results The average follow-up was 39.41 months. From all the causes, we found Lichen Sclerosus in 35%, idiopathic etiology in 29% and prior hypospadia repair in 18%. Postoperative failure occurred in 3 patients, with a final success of 82.4%. The binary regression model showed as independent risk factors ischemic cardiopathy (OR: 2.34), and the presence of Lichen Sclerosis (OR: 3.21). Conclusions The success rate with the perineal urethrostomy technique shows it to be a valid option above all when we preserve the urethral blood supply and plate. Lichen sclerosus and ischemic vascular problems are risk factors to re-stenosis. .

O tratamento bem-sucedido com fototerapia UVB banda estreita em um caso raro de líquen escleroso extragenital generalizado / Successful treatment of a rare case of widespread extragenital lichen sclerosus with narrow band UVB phototherapy

O líquen escleroso é patologia incomum acometendo tanto região genital quanto extragenital, constituindo apresentação atípica da doença. O uso de corticosteroides tópicos é considerado primeira linha para tratamento da doença genital. Lesões extragenitais são menos sensíveis a essa terapêutica. Relata-se caso de líquen escleroso em paciente do sexo feminino, de 82 anos, com envolvimento extragenital generalizado e tratamento bem-sucedido com fototerapia UVB de banda estreita. O líquen escleroso é dermatose inflamatória crônica, e as formas disseminadas da doença são raras e sem consenso em relação ao tratamento da forma extragenital. A opção pela terapia com UVB de banda estreita baseou-se nos relatos da literatura.

Lichen sclerosus is an uncommon condition affecting both the genital and extragenital region, the latter constituting an atypical occurrence of the disorder. The use of topical corticosteroids is considered the first-line treatment of this genital disease. Extragenital lesions are less sensitive to this therapy. The present study describes a case of lichen sclerosus in an 82-year-old female patient, with widespread extragenital involvement and successful treatment with narrow band UVB phototherapy. Lichen sclerosus is a chronic inflammatory dermatosis and disseminated forms of the condition are rare, with an absence of consensus on the treatment of the extragenital form. The choice for narrow band UVB therapy was based on the literature.

A Case of Lichen Sclerosus et Atrophicus Mimicking Xanthoma / 대한피부과학회지

No abstract available.

Morphea complicated by Hashimoto's thyroiditis in two sisters / 中华皮肤科杂志

Morphea complicated by Hashimoto's thyroiditis is reported in two sisters.Case 1:a 64-year-old female presented with skin rashes on the anterior neck,trunk and bilateral anterior shins for 5 years,itching skin rashes on the perineum for 4 years,and Hashimoto's thyroiditis for 9 years.Physical examination revealed grade 1 enlargement of firm thyroid gland without exophthalmos or pretibial myxedema.Dermatological examination showed pink patches on the neck and breast,sclerosis and atrophy of skin over the back,porcelain-white patches on the perineum.Histopathological findings suggested the diagnosis of morphea on the breast and lichen sclerosus et atrophicus on the perineum.Case 2:a 55-year-old female,who was the younger sister of case 1,suffered from gradual sclerosis and atrophy of skin in the left inframammary region and abdominal region for 4 years,as well as Hashimoto's thyroiditis for 3 years.Similarly,physical examination revealed grade 1 enlargement of firm thyroid gland without exophthalmos or pretibial myxedema.Hypopigmentation,sclerosis and atrophy of skin were observed in the left inframammary region,abdominal region and central back region.Histopathological examination suggested a diagnosis of morphea.According to the clinical and histopathological manifestations,periodic acid-Schiff staining and thyroid gland function test results,the 2 cases were both diagnosed as morphea complicated by Hashimoto's thyroiditis.

Liquen escleroso y atrófico: revisión de una dermatosis con múltiples manifestaciones / Lichen sclerosus and atrophic: review of a dermatosis with multiple manifestations

El liquen escleroso y atrófico (LEA) es una enfermedad inflamatoria crónica poco frecuente, de causa desconocida, con tendencia a la atrofia epidérmica y cicatrización destructiva. Predomina en mujeres, en la región anogenital, asociándose a un importante deterioro funcional y, en ocasiones, transformación maligna a carcinoma espinocelular. El tratamiento de elección es aún controvertido, siendo los corticoides tópicos de alta potencia y los inhibidores tópicos de la calcineurina los más utilizados. Se presentan cuatro casos clínicos de LEA; uno en una niña de 8 años, con una placa blanquecina atrófica localizada en tórax anterior; un segundo caso, un paciente de sexo masculino de 31 años con una placa blanquecina atrófica localizada en el glande, prepucio y cuerpo del pene; un tercer caso, un paciente de sexo masculino de 24 años con pápulas blanquecinas de 1 mm de diámetro, localizadas en el cuerpo del pene; y finalmente, una paciente de sexo femenino de 53 años con placas blanquecinas, atróficas e induradas en la axila derecha. Todos con hallazgos histopatológicos característicos que permitieron confirmar el diagnóstico de LEA. A partir de estos casos destacamos las diferentes localizaciones y edades de presentación que puede tener esta enfermedad junto con la importancia de un diagnóstico e inicio precoz del tratamiento. Esta revisión tiene como objetivo actualizar los conocimientos sobre los datos demográficos, clínicos, fisiopatológicos y terapéuticos en torno a LEA. Para ello, se realizó una búsqueda exhaustiva de la literatura utilizando los buscadores de PubMed y la Colaboración Cochrane. Resultados de la búsqueda incluyen bibliografía publicada hasta julio de 2014.

Lichen sclerosus et atrophicus (LSA) is an uncommon chronic inflammatory disease of unknown cause, prone to produce epidermal atrophy and destructive scarring. It predominates in women, in the anogenital region, usually associated with significant functional impairment and sometimes malignant transformation to squamous cell carcinoma (SCC). The treatment of choice is still controversial, with topical high potency steroids and topical calcineurin inhibitors being actually the most used. Four clinical cases are presented: one from an 8 year-old girl with a whitish atrophic plaque located on the chest; another is a male patient, aged 31, with a whitish atrophic plaque located on the glans, foreskin and body of the penis; a third case, 24 year-old male, with whitish papules of 1 mm in diameter located on the body of the penis and; finally, a female patient aged 53, with white atrophic and indurated plaques at the right axilla. All of them had characteristic histopathologic findings, confirming the diagnosis of LSA. From these cases we pretend to highlight the different locations and ages of presentation of LSA, and the importance of an early diagnosis and treatment. This review update the current understanding of the demographic, clinical, pathogenic and therapeutic data on LSA. For this, a comprehensive search of the literature was conducted using PubMed and Cochrane Library. Search results include published references until july 2014.

Carcinoma espinocelular de la vulva: caso clínico / Squamous cell carcinoma of the vulva: case report

Antecedentes: El carcinoma espinocelular (CEC) es una neoplasia epitelial maligna. La mayor parte se concentra en 4 áreas: cáncer de piel no melanoma, de cabeza y cuello, esofágico y pulmonar. El riesgo de metástasis de CEC es de 0,3-3,7 por ciento. El CEC vulvar representa aproximadamente un 3-5 por ciento de los cánceres ginecológicos. Caso clínico: Mujer de 86 años con prurito genital de larga data. Evaluada en varias oportunidades, siendo tratada como Herpes genital con valaciclovir, y Liquen Escleroso y Atrófico (LEA) con corticoides tópicos y tacrolimus con mala respuesta. Consultó por intenso prurito y nuevas lesiones vulvares. Al examen físico, destacaban 2 nódulos ulcerados en región periclitorídea izquierda e introito. La biopsia confirmó CEC bien diferenciado infiltrante. El TAC de abdomen y pelvis descartó metástasis. Se realizó radioterapia por 7 semanas. Por persistencia de la lesión, ingresó a cuidados paliativos. Dos años después la paciente está en buenas condiciones. Discusión: El CEC representa el 95 por ciento de las neoplasias vulvares. Existen 2 tipos: CEC en mujeres jóvenes, asociado a infección por virus papiloma humano de alto riesgo y CEC en mujeres mayores en relación a LEA. El 45-61 por ciento de los CEC de vulva se asocian a LEA preexistente, por lo que se recomienda el seguimiento de pacientes portadoras de LEA cada 6 meses. Conclusión: Es importante realizar biopsias de lesiones vulvares con mala respuesta a tratamiento, sobre todo si se asocia a LEA.

Background: Squamous cell carcinoma (SCC) is a malignant epithelial neoplasm. SCC can be divided into 4 groups: non-melanoma skin cancer (NMSC), head and neck, esophageal and lung cancer. The risk for metastasis of SCC is 0.3-3.7 percent. Vulvar SCC is approximately 3-5 percent of all gynecological cancers. Case report: An 86-year old woman with a history of several years of genital pruritus and many consultations for this reason, prior treatments included valacyclovir for genital herpes; topical corticosteroids and tachrolymus for lichen sclerosus et atrophicus (LEA) with poor response. She presented with pruritus and new vulvar lesions. Physical examination showed two ulcerated nodules on the left periclitorid region and the introitus. The biopsy confirmed an infiltrating well-differentiated SCC. CT-scans discarded metastases. She received 7 weeks of radiotherapy. Due to persistence of the tumor the patient entered palliative care. Two years afterwards the patient is in good condition. Discussion: SCC represents 95 percent of vulvar malignancies. There are 2 types: SCC in young women, associated with high-risk human papilloma virus infection and SCC in elder women associated to the preexistence of LEA. 45-61 percent of vulvar SCC is associated in with preexisting LEA. Patients with LEA should be followed every 6 months. Conclusion: It is important to perform biopsies of vulvar lesions that have poor response to treatment, especially if they are associated with LEA.

Morfea variedad liquen escleroso y atrófico asociada a vitiligo / Morphea lichen sclerosus et atrophicus variant associated to vitiligo

Comunicamos el caso de una mujer de 45 años, que padece de un vitiligo y una tiroiditis de Hashimoto, de once años de duración. Hace dos años, se instalan lesiones de morfea variante liquen escleroso y atrófico en el tronco, abdomen y extremidades. Esta asociación es extremadamente rara. Sugerimos la hipótesis que un fenómeno isomórfico, esté afectando las antiguas máculas de vitiligo, es más que una casualidad.

A 45-year-old woman who has suffered from a disseminated vitiligo and a Hashimoto tiroiditis for 11 years, started two years ago with a third disease, a morphea variant “lichen sclerosus et atrophicus” localized in the trunk, abdomen and limbs. The clash of both diseases is very unusual. We suggest the hypothesis of being an isomorphic phenomenon that affects the old macules of the vitiligo more than a simple coincidence of two diseases in the same person.

Case for diagnosis

Idiopathic Atrophoderma of Pasini and Pierini (IAPP) is a rare, exclusively cutaneous disease. It is more frequent in females, with incidence peak in the second and third decades of life. The etiopathogenesis remains unknown. IAPP most commonly affects the back, abdomen and proximal regions of the limbs. Lesions may be rounded, oval or circular; single or multiple. The evolution is variable and the course is initially progressive. Collagen changes such as atrophy, thinning, condensation and sclerosis may be observed in the papillary dermis. This paper describes a case of Idiopathic Atrophoderma of Pasini and Pierini with histopathologic findings.