Case for diagnosis. A pruritic eruption of keratotic papules over the face and neck

Abstract Generalized eruptive keratoacanthoma of Grzybowski is a rare variant of multiple keratoacanthomas counting with about 40 cases reported. It is a chronic and progressive disease for which none of the described therapeutic options has been entirely satisfactory. We report a case of an 83-year-old female who presented with a 3-month history of extremely pruritic, multiple, skin-coloured to erythematous to brownish, millimetric papules, with a keratotic centre. Histological examination of an incisional biopsy was consistent with the diagnosis of keratoacanthoma. The patient started acitretin 25 milligrams daily with a complete resolution of pruritus and regression of numerous lesions.

Erythrodermic psoriasis and human immunodeficiency virus: association and therapeutic challenges

Abstract: Erythrodermic psoriasis is a rare but severe type of psoriasis that may be triggered by human immunodeficiency virus infection. We describe the case of a 65-year-old male patient with chronic psoriasis who presents an exacerbation of his condition over a period of two weeks. Because of the severity of his case and subsequent need for systemic therapy, human immunodeficiency virus enzyme immunoassay was performed and tested positive. He thus began antiretroviral therapy combined with acitretin, showing good clinical response after 8 weeks of treatment. There is little evidence regarding the management of erythrodermic psoriasis associated with HIV infection, so antiretroviral therapy and systemic retinoid remain as the first-line treatment.

Clinical features of von Zumbusch type of generalized pustular psoriasis in children: a retrospective study of 26 patients in southwestern China

Abstract Background: Von Zumbusch type of generalized pustular psoriasis is a rare variant of psoriasis in children. It can occur in patients with or without psoriasis vulgaris. Objective: The aim of the study was to discuss the precipitating factors, clinical manifestations, laboratory data and therapy of von Zumbusch type of generalized pustular psoriasis in children from southwestern China and to improve the diagnosis and treatment level. Methods: A retrospective analysis was conducted for inpatients aged 14 years old or less with von Zumbusch type of generalized pustular psoriasis in our department from 2005 to 2014. Results: A total of 26 patients were included, of whom four (15.38%) had previous history of psoriasis vulgaris and one (3.85%) had previous history of psoriasis arthropathica. Mean onset age was 6.90 years. Gender distribution was equivalent. Incidence of the disease in summer and autumn was higher than that in winter and spring. Nineteen (73.08%) cases were triggered by infection, two (7.69%) cases were caused by sudden discontinuation of systemic use of corticosteroid. Twenty-four (92.31%) cases had concomitant fever. The initial lesion manifested as non-follicular sterile pustules on erythema. Sixteen patients responded well to acitretin, 11 to Tripterygium wilfordii Hook F (TwHF), two to cyclosporine, and one to methotrexate. Study limitations: This study is a retrospective one and the number of cases is small. CONCLUSION: Von Zumbusch type of generalized pustular psoriasis is a rare disease in children, infection is the most common precipitating factor, acitretin is the first-line therapy, traditional Chinese medicine TwHF also can be used.

Enfermedad de Darier. Presentación en dos hermanos / Darier's disease. Report in two brothers

La enfermedad de Darier (ED) o disqueratosis folicular fue descripta por Darier y White en 1889. Se trata de una alteración de herencia autosómica dominante, causada por la mutación del gen ATP2A2. Esta mutación interfiere en los procesos de diferenciación y crecimiento celular calcio dependientes. El diagnóstico es clínico e histopatológico. Se manifiesta con pápulas y costras eritematoparduzcas queratósicas, pruriginosas, localizadas principalmente en áreas seborreicas, pápulas blanquecinas en mucosa oral y alteraciones ungueales. Presentamos dos casos de ED en dos hermanos y realizamos una revisión de esta entidad (AU)

Darier's disease (DD) or keratosis follicularis was described by Darier and White in 1889. It is an autosomic dominant inheritance disorder caused by mutation of ATP2A2 gene. This mutation interferes in the processes of cell differentiation and calcium dependent growth. Diagnosis is clinical and histopathological. Clinical features include itchy keratotic papules and crusts, located mainly on seborrheic areas, whitish papules on oral mucosa and nail changes. Two cases of DD in two brothers is reported and a review of this entity is made (AU)

Generalized pustular psoriasis induced by systemic steroid dose reduction

Abstract: Generalized pustular psoriasis, or psoriasis of von Zumbusch, is an acute and severe clinical form of psoriasis, which usually occurs in patients with psoriasis undergoing aggravating factors. In this work, we report the case of a female patient, 70 years old, who developed generalized pustular psoriasis symptoms while reducing the dose of oral corticosteroids, improperly introduced for the treatment of alleged acute generalized exanthematous pustulosis. The differential diagnosis of generalized pustular psoriasis should be made with other pustular dermatoses, such as subcorneal pustulosis, IgA pemphigus and especially with acute generalized exanthematous pustulosis. Personal history of psoriasis and histopathological findings with psoriasiform changes and subcorneal pustule favored the diagnosis. She was treated with acitretin 30 mg / day, progressing to complete regression of the lesions.

Acrokeratosis verruciformis of Hopf - Case report

Abstract: A 54 year-old woman with a 3-year history of rheumatoid arthritis (RA) consulted us because of weight loss, fever and skin eruption. On physical examination, erythematous plaques with a pseudo-vesicular appearance were seen on the back of both shoulders. Histological examination was consistent with rheumatoid neutrophilic dermatosis (RND). After three days of prednisone treatment, the skin eruption resolved. RND is a rare cutaneous manifestation of seropositive RA, characterized by asymptomatic, symmetrical erythematous plaques with a pseudo-vesicular appearance. Histology characteristically reveals a dense, neutrophilic infiltrate with leucocitoclasis but without other signs of vasculitis. Lesions may resolve spontaneously or with RA treatment. This case illustrates an uncommon skin manifestation of active rheumatoid arthritis.

Primary localized cutaneous amyloidosis with lichen and poikiloderma-like lesions and an excellent response to systemic acitretin

Abstract: Primary localized cutaneous amyloidosis is a skin-limited amyloidosis that does not involve internal organs. It is clinically subclassified into 3 general categories and some rare variants. However, there is considerable overlap within the classification. Though there are a variety of therapeutic measures, the treatment is often unsatisfactory, particularly when the disease is severe and extensive. We describe a rare case of primary localized cutaneous amyloidosis with lichen and poikiloderma-like lesions that showed an excellent response to systemic acitretin.

NAPSI utilization as an evaluation method of nail psoriasis in patients using acitretin / Utilização do NAPSI como instrumento de avaliação na psoríase ungueal em pacientes com uso da acitretina

BACKGROUND: assessment instruments of nail psoriasis have been published in literature as means to standardize the previous subjective assessments of authors. OBJECTIVES: Using Nail Psoriasis Severity Index for evaluation in patients with plaque psoriasis during treatment with acitretin. METHODS: Thirty volunteers with plaque psoriasis were selected for the study. Twenty patients (8 women and 12 men) completed the study. The initial dose of acitretin was 0.3 mg/kg/day for 30 days and was later raised to 0.5 mg/kg/day. Nail Psoriasis Severity Index was collected in the first evaluation, after 2 and 4 months. Nails of both hands were evaluated. RESULTS: nail lesions were present in all patients at first evaluation. The initial Nail Psoriasis Severity Index median was 20 and the final score 20.5 (2.5% of worsening at the end of the study). No statistically significant difference between the three evaluations was found (X2 = 0.8084, GL = 2, p = 0.6657). Seven patients worsened in the final score. Three patients improved 50% of the initial Nail Psoriasis Severity Index and only one had an improvement of 75%. Linear correlation showed a weak association between the improvement percentage in PASI and Nail Psoriasis Severity Index (r = 0.105, F = 2.12, p = 0.162). CONCLUSION: The method was easy and of rapid execution while potentially bringing information about changes in nail plate and matrix during treatment. The Nail Psoriasis Severity Index does not quantify the existing lesions and might not have the sensitivity to detect small changes.

FUNDAMENTOS: instrumentos de avaliação da psoríase ungueal têm sido publicados na literatura como meio de padronizar as avaliações antigamente subjetivas dos autores. OBJETIVOS: utilizar índice de Gravidade da Psoríase Ungueal ou Nail Psoriasis Severity Index para avaliação em pacientes portadores de psoríase em placas durante o tratamento com acitretina. MÉTODOS: trinta voluntários portadores de psoríase em placas foram selecionados para o estudo. Vinte deles ( 8 mulheres e 12 homens) completaram o estudo. A dose inicial da acitretina foi de 0,3mg/kg/dia por 30 dias sendo elevado posteriormente para 0,5mg/kg/dia. Utilizou-se a avaliação do Nail Psoriasis Severity Index na primeira avaliação, após 2 e 4 meses avaliando-se as unhas de ambas as mãos. RESULTADOS: lesões de unhas estavam presentes em todos os pacientes na primeira avaliação. A mediana inicial do Nail Psoriasis Severity Index foi de 20 e o escore final 20,5 (2,5% de piora ao final do estudo), sem diferença estatística significativa entre as três (X2=08084, GL=2, p=0,6657). Sete pacientes pioraram no escore final. Três pacientes obtiveram melhora de 50% do Nail Psoriasis Severity Index inicial em relação ao final e apenas 1 obteve melhora de 75 . A correlação linear simples mostrou fraca associação entre a porcentagem de melhora do PASI e do Nail Psoriasis Severity Index (r=0,105, F=2,12, p=0,l62). CONCLUSÕES: o método se mostrou de fácil e de rápida execução, podendo trazer informações sobre as alterações de lâmina e matriz de um modo global. O Nail Psoriasis Severity Index não quantifica as lesões existentes isoladamente, podendo não ter a sensibilidade de detectar pequenas alterações.

Pitiriasis rubra pilaris eritrodérmica: algo para no olvidar / Erythrodermic pityriasis rubra pilaris

La Pitiriasis Rubra Pilaris (PRP) es una dermatosis papuloescamosa crónica, de etiología desconocida. Se caracteriza por la presencia de pápulas foliculares hiperqueratósicas que coalescen formando placas eritematoescamosas, dejando islotes de piel sana entre las lesiones. Puede llegar a una eritrodermia. Se clasifica en base a la edad de presentación, características morfológicas, evolución y pronóstico. Existen múltiples opciones de tratamiento descritas en la literatura, siendo los retinoides sistémicos el tratamiento de primera línea en estos pacientes. Presentamos dos casos de pacientes con PRP eritrodérmica tratados exitosamente con Acitretín y revisión de la literatura a la fecha.

Pityriasis Rubra Pilaris is a chronic inflammatory dermatosis of unknown etiology, characterized by the presence of multiple follicular papules that coalesce into large erythematous or salmon colored plaques with islands of non-involved skin between them. It can eventually evolve into erythroderma. Descriptions and therapeutic experiences are mainly based on case reports. Today retinoids have become de first line treatment in these patients. We present two cases of erythrodermic PRP treated successfully with Acitretin and an updated review of the literature.

Hiperceratose epidermolítica: um seguimento de 23 anos de uso de retinoides orais / Epidermolytic hyperkeratosis: a follow-up of 23 years of use of systemic retinoids

A hiperceratose epidermolítica é uma forma de ictiose geralmente resistente a tratamentos tópicos. Relata-se um caso de paciente feminina , em acompanhamento na dermatologia desde 1978, com diagnóstico de hiperceratose epidermolítica. Foi tratada inicialmente com queratolíticos, vitamina A oral, ácido tartárico e emolientes tópicos, porém sem melhora no quadro clínico, já que não haviam disponíveis outros tratamentos na época. Em 1986, com o advento dos retinóides orais, foi introduzido o etretinato, e em 1998, foi substituído pelo acitretin, apresentando excelente resposta terapêutica. No momento a paciente está em uso de acitretin 25 mg/dia, completando 23 anos de uso de retinóides orais, com mínimos efeitos adversos e melhora significativa na qualidade de vida.

Epidermolytic hyperkeratosis is a form of ichthyosis normally resistant to topical treatments. Female patient monitored since 1978 diagnosed with epidermolytic hyperkeratosis. Clinical examination showed generalized hyperkeratosis and scaling. Given that no other treatments were available at the time, the patient was initially treated with keratolytic, systemic vitamin A and moisturizers, with no improvement. In 1986, with the development of oral retinoids, etretinate was introduced. In 1998 this was replaced by acitretin. The patient is receiving 25 mg/day after 23 years of using oral retinoids. Significant improvement of the condition and patient's quality of life has been noted.

Líquen plano hipertrófico disseminado: relevante resposta à acitretina / Disseminated hypertrophic lichen planus: relevant response to acitretin

O líquen plano hipertrófico é uma variante do líquen plano, com pronunciada hiperplasia epidérmica em resposta à coçadura persistente. Clinicamente, caracterizam-se por placas hiperceratósicas, simétricas, de coloração cinza-violácea, com predileção pela região pré-tibial. O prurido intenso, a refratariedade aos tratamentos convencionais e a possibilidade de associação de um carcinoma epidermoide às lesões de longa duração impõem um tratamento eficaz. Os corticoides são considerados o tratamento de primeira linha e podem ser aplicados topicamente ou empregados de forma sistêmica. Outras modalidades terapêuticas propostas são a fototerapia com UVB-NB ou PUVA, imunossupressores e retinoides sistêmicos, com destaque para a acitretina. Relatamos um caso com apresentação clínica exuberante e excelente resposta à acitretin, totalizando um seguimento de nove meses.

Hyperthrofic lichen planus is considered a variant of lichen planus with marked epidermal hyperplasia in response to persistent itch. It is clinically, characterized by symmetric hyperkeratotic plaques, of purplish-grey color, often located in the pretibial region. Intense pruritus, refractoriness to conventional treatments and the possibility of association of the long-term injuries with squamous cell carcinoma requires an effective treatment. The first-line treatment is corticosteroids which can be applied either topically or systemically. Other therapeutic modalities proposed are: NB-UVB phototherapy or PUVA, immunosuppressive drugs and systemic retinoids, notably acitretin. We report a case with exuberant clinical presentation of hyperthrofic lichen planus with excellent response to acitretin after nine months of treatment.

Queratose liquenoide crônica: relato de caso / Keratosis lichenoides chronica: case report

A queratose liquenoide crônica ou doença de Nekam é uma dermatose mucocutânea rara da queratinização, com curso crônico e progressivo, que acomete geralmente indivíduos entre 20 e 40 anos. Existem, aproximadamente, 70 casos descritos na literatura. Devido à raridade desta dermatose e à ausência de tratamento efetivo, é uma doença de difícil manejo. No caso em questão, apresentamos um paciente de 42 anos com pápulas violáceas, hiperqueratósicas, algumas confluentes, com aspecto linear, rendilhado e em placas, localizadas no tronco e membros há cinco anos. Lesões aftoides na cavidade oral e úlceras rasas na genitália também faziam parte do quadro. O anatomopatológico foi bastante sugestivo de queratose liquenoide crônica. Introduziu-se tratamento com acitretina e dapsona, havendo melhora parcial do quadro.

Keratosis lichenoides chronica or Nekam's disease is a rare mucocutaneous dermatosis characterized by keratinization. It is chronic and progressive usually affecting individuals aged 20-40 years. Around 70 cases have been reported in the literature. Due to the rarity of this condition and the lack of effective treatment, it is a difficult disease to manage. In the case described below we present a 42-yearold patient with violaceous and hyperkeratotic papules in linear, reticular or plaque form, located on the trunk and limbs for five years. Aphthous lesions in the oral cavity and shallow ulcers on the genitalia also formed part of the clinical manifestation. Pathologic examination was suggestive of keratosis lichenoides chronica. Acitretin and dapsone was introduced and the lesions partially improved.

Granuloma actínico de O'Brien: apresentação clínica exuberante / O'Brien's actinic granuloma: an unusually extensive presentation

O granuloma actínico de O'Brien é uma doença cutânea rara. Há muitas controvérsias na sua caracterização, podendo representar uma entidade nosológica distinta ou uma forma de granuloma anular em áreas expostas. A patogênese é desconhecida; no entanto, acredita-se que a radiação solar seja um fator desencadeante. Os autores relatam o caso de um paciente de 78 anos, branco, que apre sentava há 10 anos pápulas eritematosas e infiltradas na fronte e hemiface à esquerda. O diagnóstico de granuloma actínico de O'Brien foi estabelecido a partir do exame histopatológico, uma vez que o quadro clínico exuberante apresentado pelo paciente diferia do de casos anteriormente observados.

O'Brien's actinic granuloma is a rare skin disease. Controversy continues over whether it should be considered a specific condition or a form of granuloma annulare located in sun-exposed areas. Its pathogenesis is unknown; however, the most widely accepted hypothesis suggests that solar radiation is the triggering factor. This paper describes the case of a 78-year old, fair-skinned male, who presented with a 10-year history of an infiltrate of annular erythematous papules on his forehead and left malar region. The diagnosis of O'Brien actinic granuloma was established from histopathology, since the clinical condition of the patient was extensive, unlike cases reported in the literature.

Ichthyosis follicularis, alopecia and photophobia syndrome (IFAP): report of the first case with ocular and cutaneous manifestations in Brazil with a favorable response to treatment / Síndrome de ictiose folicular, alopécia e fotofobia (IFAP): relato do primeiro caso com acometimento ocular e cutâneo do Brasil e da resposta favorável ao tratamento

Ichthyosis follicular, alopecia, and photophobia (IFAP) syndrome is a rare disease, with possible X-linked mode of inheritance. The patient presented with ocular findings of photophobia, corneal scarring and erosions, superficial and deep corneal vascularization and myopia. He was treated with artificial tears and punctal occlusion with small improvement of photophobia. After three months using systemic retinoid (Acitretina) and posterior amniotic membrane transplantation in the left eye, there was a significant improvement of photophobia, corneal erosions and neuropsychomotor development.

A síndrome de ictiose folicular, alopecia e fotofobia (IFAP) é uma doença rara, com possível modo de herança ligado ao cromossomo X. O paciente apresentou achados oftalmológicos de fotofobia, cicatrizes e erosão corneanas, neovascularização superficial e profunda da córnea e miopia. Foi iniciado uso de lubrificantes e oclusão do ponto lacrimal com discreta melhora da fotofobia. Após uso de retinóide sistêmico (Acitretina) por três meses e posterior transplante de membrana amniótica no olho esquerdo apresentou melhora importante da fotofobia, das erosões corneanas e do desenvolvimento neuropsicomotor.