Acroqueratoelastoidosis: reporte de un caso clínico / Acrokeratoelastoidosis: reportof a clinical case

Resumen La acroqueratoelastoidosis es una rara genodermatosis de herencia autosómica dominante, familiar o esporádica, siendo esta última la forma más frecuentemente reportada en la literatura. Fue descripta por el dermatólogo de origen brasileño Oswaldo Costa en el año 1953. Así las acroqueratodermias marginales son un subgrupo de queratodermiaspalmoplantares caracterizadas por la presencia de pápulas y placas queratósicas, con una disposición lineal, que asientan sobre el margen de transición entre la piel dorsal y palmar o plantar.Suelen iniciarse en la infancia, en la adolescencia o en la vida adulta temprana y tienen un curso crónico. Su diagnóstico diferencial con el resto de las acroqueratodermias es un gran desafío, siendo el hallazgo histológico de elastorrexis lo primordial para su correcto diagnóstico. Presentamos el caso de una mujer de 22 años con un cuadro compatible clínica e histopatológicamente con Acroqueratoelastoidosis.

Abstract Acrokeratolastoidosis is a rare genodermatosis of dominant autosomal, familial or sporadic inheritance, the latter being the most frequently reported form in the literature. It was described by the Brazilian dermatologist Oswaldo Costa in 1953. It is characterized by the presence of multiple hyperkeratotic papules, usually asymptomatic, located in the marginal area of the hands, feet or both. It usually begins in childhood, adolescence or early adult life and has a chronic course. Its differential diagnosis with the rest of the acrokeratosis is a great challenge, being the histological finding of elastorrexis the primary for its correct diagnosis. We present the case of a 22-year-old woman with a clinical and histopathology compatible with Acroqueratoelastoidosis.

Prolonged luteal phase support with progesterone may increase papules and plaques of pregnancy frequency in pregnancies through in vitro fertilization

Abstract Background: Pruritic urticarial papules and plaques of pregnancy development may have a strong relationship with hormone treatments during in vitro fertilization and hormonal changes during pregnancy. Objectives: The aim of this study was to evaluate and compare the frequency of papules and plaques of pregnancy and related factors in in vitro fertilization pregnancies and spontaneous pregnancies. Methods: In this study, 517 in vitro fertilization pregnancies and 1253 spontaneous pregnancies were retrospectively reviewed for papules and plaques of pregnancy frequency. The diagnosis of papules and plaques of pregnancy was performed by referral to the dermatology department and according to the typical clinical manifestations of the disease. Results: The papules and plaques of pregnancy was more common in all in vitro fertilization pregnancies (including single pregnancies) than in spontaneous pregnancies. Age, Rh positivity, mother weight gain, onset of disease during gestation, duration of disease, birth weight and the frequency of male fetus were similar between the two groups (p > 0.05). The rate of multiple pregnancies was higher in in vitro fertilization pregnancies with papules and plaques of pregnancy than in vitro fertilization pregnancies without papules and plaques of pregnancy (p < 0.001). Duration of progesterone treatment was also significantly longer in in vitro fertilization pregnancies with papules and plaques of pregnancy compared to in vitro fertilization pregnancies without papules and plaques of pregnancy (p < 0.001). Study limitations: The limitations of the study were the retrospective and single-centered design. Conclusion: The results of this study indicate that increased progesterone dosage or prolonged treatment may play a role in the pathogenesis papules and plaques of pregnancy.

Liquen Plano Pigmentado:a propósito de un caso / Flat LichenPigmented:Clinical case

RESUMEN El Liquen Plano Pigmentado es una dermatosis crónica frecuente de etiología aún desconocida, caracterizada clínicamente por la presencia de pápulas purpúreas, poligonales, pruriginosasdistribuidas en forma simétrica. ElLiquen Plano Pigmentado representa una de todas las variantes clínicas, cuya característica histológica distintiva es la incontinencia de pigmento melánico. Su curso es autolimitado pues se resuelve en un período de meses a años, pero puede persistir indefinidamente. Existen múltiples opciones de tratamiento, que incluyen corticoides tópicos, intralesionales y sistémicos, retinoides, fototerapia, inmunomoduladores tópicos y, en casos graves o resistentes al tratamiento, la ciclosporina, metotrexato. La dermatoscopía es una técnica auxiliar de diagnóstico no invasivaque permite incrementar la certeza diagnóstica en diversasenfermedades cutáneas; en el caso del Liquen Plano Pigmentado se observa la lesión clásica que consiste en una pápula poligonal aplanada, de tonalidad eritemato-violácea que suele tener finas escamas adherentes en la superficie,denominadasestrías de Wickham, queconsisten en estructuras polimórficas blanquecinas y que corresponden histológicamente a zonas de ortoqueratosis.Es una herramienta de gran utilidad para el diagnóstico de estas lesiones.

ABSTRACT The Pigmented Flat Lichen is a frequent chronic dermatosis of etiology still unknown, clinically characterized by the presence of purpuric, polygonal, pruritic papules distributed symmetrically. The Pigmented Flat Lichen represents one of all clinical variants, where the distinctive histological feature is melanin pigment incontinence. Its course is self-limited, it resolves over a period of months to years, but it can persist indefinitely. There are multiple treatment options that include topical, intralesional and systemic corticosteroids, retinoids, phototherapy, topical immunomodulators and in severe or resistant cases to treatment, cyclosporine, methotrexate. Dermatoscopy is a non-invasive diagnostic auxiliary technique that allows accelerating diagnostic certainty in various skin diseases; in the case of the Pigmented Flat Lichen, the classic lesion is observed, consisting of a flattened polygonal papule, with an erythematous-violet hue that usually has fine adherent scales on the surface, called Wickham striae, which consists of whitish polymorphic structures and that corresponds histologically to areas of orthokeratosis. It is a very useful tool for the diagnosis of these lesions.

pt / Presentación clínica de la enfermedad de darier lineal. relato de caso / Clinical presentation of linear darier's disease. a case report

RESUMEN: La enfermedad de Darier es una genodermosis autosómica dominante, caracterizada por acantolisis y queratinización anormal, con hallazgos muco-cutáneos característicos, como pápulas queratóticas en el tronco superior y eritroniquismo longitudinal y que posee una forma localizada, rara, correspondiente a un mosaico genético. Esta forma de presentación envuelve la superficie de la piel de forma lineal, zosteriforme, irregular o con otro formato localizado, siendo el tronco y los miembros los lugares más afectados. En este caso de la enfermedad de Darier, su presentación fue unilateral, siguiendo las líneas de Blaschko, con superficie hipertrófica, configurando la enfermedad de Darier lineal. El paciente presentaba múltiples placas hipocrómicas a lo largo del miembro inferior derecho, de la raíz del muslo hasta la matriz ungueal del primer dedo. En la región inguinal derecha, hubo confluencia de las pápulas, formando una placa condilomatosa, grisácea y pruriginosa. Optamos por realizar un examen histopatológico de una pápula de la pierna y de la placa inguinal.

SUMMARY: Darier's disease is an autosomal dominant genodermatosis, characterized by acatholysis and abnormal keratinization, with characteristic mucocutaneous findings, such as keratotic papules in the upper trunk and longitudinal erythronychia. There is a localized rare presentation that corresponds to a genetic mosaicism, which involves the surfaceof the skin in a linear, zosteriform, irregular or other localized arrangement, being the trunk and limbs the most affected areas. We report amosaicism of Darier's disease, whose presentation was in a linear, unilateral pattern, following the lines of Blaschko, with localized hypertrophic formation, configuring the Linear Darier's disease. The patient presented with multiple hypochromic planes along the right lower limb, from the thigh root to the nail matrix of the first toe. In the right inguinal region, there was confluence of the papules, forming a condylomatous, grayish and pruritic plaque. We chose to perform histopathological examination from a papule on the leg and the inguinal plate. The therapy was directed to improve the patient's quality of life.

Liquen amiloideo: diagnóstico diferencial de pápulas agminadas en piernas / Lichen amyloid as differential diagnosis of grouped papules in the legs

El liquen amiloideo es un tipo de amiloidosis cutánea primaria localizada, de etiología desconocida. Generalmente afecta a adultos, siendo más frecuente en ciertos grupos étnicos. Clínicamente se manifiesta por pápulas pruriginosas, frecuentemente localizadas en miembros inferiores. Presentamos el caso de una mujer de 50 años, con un historial de dos años caracterizado por pápulas agminadas en piernas, que tras la realización de una biopsia se diagnosticó liquen amiloideo.

Amyloid lichen is a type of localized primary cutaneous amyloidosis of unknown etiology. It usually affects adults being more frequent in certain ethnic groups. Clinically it is manifested by pruritic papules frequently located in lower limbs. We present the case of a 50-year-old woman, with a two-year history characterized by papules agminated in the legs that diagnosed amyloid lichen after biopsy.

Three cases of dermatomyositis presenting with inverse Gottron's papules / 中华皮肤科杂志

Three cases of dermatomyositis presenting with inverse Gottron's papules were reported.Of the 3 patients,there were 2 males and 1 female aged 43,41 and 46 years respectively,whose disease durations were 1,6,7 months respectively.Inverse Gottron's papules manifested as papules overlying the palmar aspect of the metacarpal and interphalangeal joints,which were arranged in a linear pattern in 2 cases.Of the 3 cases,1 presented with interstitial pneumonia,3 with myositis,and 2 showed negative anti-nuclear antibody test.Histopathological examination of inverse Gottron's papules in 1 case revealed focal liquefaction degeneration of basal cells and perivascular infiltration with inflammatory cells,especially lymphocytes,in the superficial dermis.The 3 patients received oral glucocorticoids and immunosuppressive agents in the early period of treatment.After 3-month treatment,clinical symptoms including muscle weakness and muscle pain were improved evidently,but no obvious improvement in inverse Gottron's papules was observed.After 1-year treatment,these papules on the palmar aspect were markedly relieved in 1 case.

A Case Report of Acne Agminata

Granulomatous facial skin lesions are a rare and challenging clinical problem. Differential diagnoses include cutaneous tuberculosis, sarcoidosis, granulomatous rosacea and acne agnimata. We reported a case of acne agminata presented with granulamatous facial papules.

Rash and weakness - A case of juvenile dermatomyositis

Juvenile dermatomyositis (JDM) is a well-known but uncommon paediatric rheumatological condition. It can be missed early in the disease manifestation if not thought of in patients presenting with atypical rash and weakness. We present a case of a 5-year-old girl who developed JDM over a span of 8 weeks before a diagnosis was made. This case demonstrates the nuances of how a paediatric patient’s presentation can differ from adults’. A high index of suspicion and early appropriate referral to paediatric rheumatology are paramount for successful management and good prognosis of the condition.

Speckled pigmentation and palmoplantar keratoses leading to the mass detection of chronic arsenic poisoning

@#<p style="text-align: justify;">Arsenic is a known human carcinogen and skin manifestations are the earliest and most specific markers of chronic arsenic poisoning. A 43-year-old man from Luzon presented at the Section of Dermatology with a one-year history of hyperkeratotic papules and plaques on the palms and soles. Numerous round hypopigmented macules were scattered on the upper back. Initial 24-hour urine arsenic level was elevated at 288mcg/liter. The patient underwent successful chelation with N-acetylpenicillamine and the palmoplantar keratoses were treated with cryotherapy and topical 20% salicylic acid in white petrolatum. In cooperation with the Department of Health, a comprehensive health and environmental assessment was conducted in the affected communities. This case highlights the role of dermatologists in the diagnosis and management of this public health problem.</p>

A Case of Atypical Adult-onset Still's Disease That Presented with Unique Histopathologic Findings / 대한피부과학회지

Adult-onset Still's disease (AOSD) is an uncommon disorder characterized by fever, polyarthralgia, elevated white blood cell count, and maculopapular rash, the histologic features of which have not been well known. A 54-year-old woman presented with fever, arthritis, and persistent pruritic lichenified rash. These cutaneous lesions showed unique histological features such as dyskeratosis with a peculiar, distinctive distribution in the upper epidermis and cornified layers with hyperkeratosis. In addition, dermal mucin deposition was increased, with inflammation and minimal fibroblast proliferation. The patient was diagnosed with AOSD according to the diagnostic criteria of Yamaguchi. Herein, we present an atypical form of AOSD showing distinctive clinical and pathological manifestations.

Clinical features of multicentric reticulohistiocytosis / 中华风湿病学杂志

Objective To study the clinical profile of patients with multicentric reticulohistiocytosis (MRH).Methods The clinical manifestations,image and histologic characteristics of ten patients with MRH were collected,and the relevant literatures were reviewed.Results Seven cases were women.The median (quartile) age was 46.5 (44.5-55.3) years.Clinical symptoms included papulonodular lesions on the skin (ten cases),erosive polyarthritis (ten cases),myositis (four cases),lung nodules (three cases),larynx nodules (one case) and serositis (one case).Biopsy demonstrated infiltration of multinucleated giant cells with CD68 positive and glassy cytoplasm.All of the ten patients were treated with corticosteroids and disease-modifying antirheumatic drugs,skin lesions of seven patients were ameliorated and arthritis was improved in three patients.Conclusion MRH is a systemic disease that has characteristic clinical manifestations and unique histological findings.Deep understanding of this disease can avcid.isdiagnose.Further studies are needed to explore effective treatment.

Disseminated Papules and Pustules with Lymphadenitis after Bacillus Calmette-Guerin Vaccination in an Infant / 대한피부과학회지

Bacillus Calmette-Guerin (BCG) is the live attenuated strain of Mycobacterium bovis that produces resistance to tuberculosis (TB) infection. BCG vaccination may induce various dermatologic complications. A 3-month-old infant who had BCG vaccination a month after birth developed erythematous papules and pustules on the trunk and both extremities. The biopsy from the left leg showed a granulomatous lesion. Acid fast staining and nested TB polymerase chain reaction (TB-PCR) were negative. A tuberculin skin test and an interferon-gamma release assay also revealed a negative result. A painful mass was noted on the left axillary area with a positive-nested TB-PCR and surgical excision was performed. The skin eruptions improved after 2 months of anti-tuberculosis medication. Herein, we report a case of disseminated papules and pustules with lymphadenitis after BCG vaccination.

Sarcoma histiocítico cutâneo: neoplasia rara e de difícil diagnóstico / Cutaneous histiocytic sarcoma: a rare and difficult to diagnose neoplasm

Os sarcomas cutâneos são tumores raros, altamente agressivos e de diagnóstico muitas vezes difícil para o dermatologista. Apresentamos o caso de uma mulher branca, de 78 anos, com nódulo eritêmato-violáceo de crescimento rápido em ombro esquerdo, descrito inicialmente como melanoma amelanótico à histologia, conduzida como tal e, após estudo imuno-histoquímico, ausência de marcadores para lesões melanocíticas e expressão de CD45RB (antígeno leucocitário humano) e CD68 (proteína lisossomal) possibilitaram diagnóstico final de sarcoma histiocítico.

Rosácea: mais que um rosto vermelho / Rosacea: more than a red face

A rosácea é uma doença cutânea crônica comum, de etiologia desconhecida, que ocorre mais comumente em indivíduos de meia-idade. As manifestações cutâneas incluem eritema facial transitória ou persistente, telangiectasia, edema, pápulas, pústulas e que são geralmente confinados à porção central da face. Os medicamentos tópicos e sistêmicos são eficazes para o tratamento da rosácea. Casos persistentes podem ser tratados por via oral com metronidazol, tetraciclina ou isotretinoína. Este artigo relata um paciente com rosácea tratado com isotretinoína oral.

Two Cases of Pruritic Urticarial Papules and Plaques of Pregnancy in the Postpartum / 대한피부과학회지

Pruritic urticarial papules and plaques of pregnancy (PUPPP), also known as a polymorphic eruption of pregnancy, is the most common dermatosis of pregnancy. Typically, PUPPP starts with erythema and urticarial papular eruption in the abdominal striae and may extend to the buttocks and thighs. It usually evolves in the third trimester and resolves rapidly in the postpartum. It is not usually recurrent in subsequent pregnancies. We report herein two patients with postpartum PUPPP.

Pruritic Urticarial Papules and Plaques of Pregnancy: Clinical Experience with 22 Patients / 대한피부과학회지

BACKGROUND: Pruritic urticarial papules and plaques of pregnancy is a common benign dermatosis of pregnancy. It occurs mainly in primigravidas in the third trimester, it resolves spontaneously or with delivery, and it is usually responsive to topical treatment. The morphological spectrum of pruritic urticarial papules and plaques of pregnancy includes (1) urticarial papules and plaque (type I), (2) non-urticarial erythematous patches that are discrete or confluent and they are surrounded by tiny papules or vesicles, or clusters or sheets of 1-mm to 2-mm erythematous papules (type II) and (3) a combination of these features (type III). OBJECTIVE: This study evaluated our clinical experience with 22 Korean patients with pruritic urticarial papules and plaques of pregnancy. METHODS: From June 1997 to August 2008, 22 patients with pruritic urticarial papules and plaques of pregnancy were evaluated. The clinical manifestations, the lesion distribution and the obstetric and pediatric findings were retrospectively investigated with using the patients' medical records and clinical photographs. RESULTS: There were 9, 11 and 2 patients with types I to III, respectively. Many patients (n=14) were primigravida and the mean time of onset was a gestational age of 29.8 weeks. There were no pediatric problems such as intra-uterine growth retardation, or a small or large size for the gestational age.Most of the patients (n=20) were treated with topical corticosteroid or antihistamine; there was one case of recurrent pruritic urticarial papules and plaques of pregnancy. CONCLUSION: Pruritic urticarial papules and plaques of pregnancy usually occur in primigravidas, but this can also occur in multigravida patients. The number of patients with pruritic urticarial papules and plaques of pregnancy and who had obstetric obesity, a multiple pregnancy or pregnancy-induced hypertension was fewer than we expected. Pruritic urticarial papules and plaques of pregnancy can be divided into three categories, although this is rather redundant because the disease prognosis and treatments are not different according to the type.

Pápulas umbilicadas diseminadas asociadas a infección por VIH-sida / Disseminated umbilicated papules associated with HIV infection/AIDS

Se presenta el caso de un varón de 29 años, con infección por VIH/sida, quien presenta múltiples pápulas amarillentas, de 2 a 4 mm, no dolorosas, muchas de ellas umbilicadas, diseminadas en todo el cuerpo. Se plantean diagnósticos de enfermedades infecciosas, neoplásicas, y reactivas.

We report the case of a man, 29 years old, with HIV Infection/AIDS, who presents numerous yellowish papules, many of them umbilicated, not painful, disseminated in the whole body surface. Differential diagnosis include infections, tumours, and reactive processes.

A Case of Familiar Painful Piezogenic Pedal Papules / 대한피부과학회지

We report a case of painful pizogenic pedal papules in a 10-year-old female patient who had developed tense, skin-colored papules with pain on both heels when weight was placed on the feet. Histopathologic findings showed subcutaneous fatty tissue protruding into the lower dermis and a basophilic degenerative change of part of the collagen in the dermis. Her father also had the same skin lesions on his both heels.

A Case of Pruritic Urticarial Papules and Plaques of Pregnancy (PUPPP) Syndrome Supposedly Caused by the Paternity in Late Pregnancy / 대한주산의학회잡지

Pruritic Urticarial Papules and Plaques of Pregnancy (PUPPP) is a common dermatosis of pregnancy which usually occurs in the third trimester and generally in that of primigravida. Clinical feature is characterized by tiny erythematous papules which soon coalesce to form large, erythematous plaques. It generally occurs in abdomen, buttocks, thighs and upper inner arms. Since 1979 when Lawley et al. first described and reported PUPPP, there has been a lot of reports on PUPPP but the etiology is still not known. Many etiologic factors were reported but paternity as an etiologic factor was rarely reported. We had a very rare case which showed paternity could be one of the possible etiologic factors and we would like to share our case though this report.

A Case of Primary Cutaneous CD30+/ALK- Anaplastic Large Cell Lymphoma Presenting with Nodules and Numerous Eruptive Papules / 대한피부과학회지

Primary cutaneous CD30-positive, anaplastic large cell lymphoma (ALCL) is a rare cutaneous peripheral T cell lymphoma with a favorable prognosis. ALCL is characterized by a solitary or localized skin tumor composed of sheets of atypical lymphoid cells with pleomorphic or anaplastic nuclei. Herein we report a 67-year-old woman presented with tender nodules and severely pruritic numerous eruptive papules. Histopathologic examination revealed dense non-epidermotrophic infiltrates of atypical large cells with round, oval or irregularly shaped nuclei and abundant cytoplasm. Immunohistochemical studies demonstrated that most lymphoid cells in the dermis and subcutis were positive for CD30 and CD45RO, and negative for CD20 and CD56.