Perinevic dermatosis neglecta: clinical and dermoscopic description

Abstract: Perinevic dermatosis neglecta is an underestimated skin condition usually affecting children and adolescents, characterized by the appearance of dirt-like brownish pigmentation around a preexisting nevus and often a matter of concern for parents. We describe the clinical and dermoscopic findings in a case of perinaevic dermatosis neglecta and discuss the possible etiology of this phenomenon.

Frontal fibrosing alopecia and extrafacial lichen planus pigmentosum in a caucasian woman

Abstract We present the case of a 72-year-old Caucasian woman with frontal fibrosing alopecia of one year's duration. Eighteen months later, she presented with sudden hyperpigmentation on the submental area, neck and upper chest. Histopathological examination was consistent with lichen planus pigmentosus. This is the report of a unique case of a FFA and extrafacial lichen planus pigmentosus.

Coexistence of nail lichen planus and lichen planus pigmentosus

Abstract We describe a fifty-six-year old, Afro-descendent female patient showing dystrophy of her twenty nails and hyperchromic, asymptomatic macule on her face. Histopathological examination of the macule showed vacuolization of the basal layer, melanophages in the superficial dermis and lymphoplasmocytic inflammatory infiltrate. Nail biopsy revealed orthokeratotic hyperkeratosis and lichenoid inflammatory infiltrate. Lichen planus pigmentosus is an uncommon variety of lichen planus. It is characterized by typical hyperpigmented macules on the face and neck. Nail changes might be present in 10% of lichen planus cases, but no associations with lichen planus pigmentosus have been described. We report a case of lichen planus in twenty nails associated with lichen planus pigmentosus on the patient's face.

Posible asociación autoinmune entre síndrome de Laugier-Hunziker y síndrome de Sjögren: reporte de un caso y revisión de la literatura / Laugier-Hunziker syndrome in a patient with Sjögren’s syndrome: report of one case

Laugier-Hunziker syndrome is a rare benign idiopathic condition characterized by acquired macular pigmentation of lips and buccal mucosa, often accompanied with melanonychia. The main concern with this condition is to rule out other differential diagnosis with systemic repercussions and similar hyperpigmentation patterns, such as Peutz-Jeghers syndrome, adrenal insufficiency and melanoma. We report a 58-year-old female with a 20-year history of Sjögren’s syndrome, presenting with melanonychia and hyperpigmentation in the buccal mucosa. She had no relevant medication history and is a non-smoker. The patient denied any other symptoms. The histopathology confirmed the diagnosis of Laugier-Hunziker syndrome.

Enfermedad de Dowling-Degos: comunicación de caso en un adolescente / Dowling-Degos disease: case report in an adolescent

La enfermedad de Dowling-Degos es un raro trastorno de la pigmentación, con comportamiento benigno y patrón autosómico dominante. Se comunica el caso de un adolescente sin antecedentes personales de importancia que acude al servicio de Dermatología por máculas hipo e hiperpigmentadas que se distribuían en grandes pliegues y en zonas de presión, de 2 años de evolución. Por los datos clínicos y los estudios complementarios se determinó el diagnóstico de enfermedad de Dowling-Degos.

The Dowling-Degos disease is a rare disorder of pigmentation with benign behavior and autosomal dominant pattern. This paper reports the case of a teenager with no personal history of importance that was referred to the Dermatology Department for presenting hypo and hyperpigmented spots distributed in the large folds and pressure zones, of 2 years of evolution. Considering clinical data and additional studies the diagnosis of Dowling-Degos disease was done.

Hiperplasia suprarrenal congenita. Reporte de caso / Congenital Adrenal Hyperplasia. Case Report

La Hiperplasia Suprarrenal Congénita (HSC) es una enfermedad endocrina genética. En el 90% de los casos es causada por mutaciones en el gen CYP21A2, que codifica la enzima esteroide adrenal 21-hidroxilasa (21-OH) implicada en la esteroidogénesis. Esto provoca hipersecreción de la hormona adrenocorticotropa (ACTH), llevando a la producción anormal- mente elevada de andrógenos suprarrenales. Se trata de un recién nacido con genitales ambiguos caracterizados por hipertrofia de clítoris, con la piel hiperpigmentada en el área genital, fusión labio escrotal en línea media y seno urogenital abajo del falo o clítoris de aproximadamente 1 mm de diámetro. No se palparon gónadas. El estudio sonográfico reportó glándulas suprarrenales dentro de los límites normales, con útero de forma y tamaño normal. No se observó presencia de testículos. El diagnóstico oportuno permitió la instauración del tratamiento en los primeros días de vida con la prevención subsecuente de complicaciones...(AU)

Caso para diagnóstico / Case for diagnosis

A doença de Dowling-Degos é uma genodermatose rara, caracterizada principalmente por hiperpigmentação reticular progressiva de áreas flexurais. Apesar de apresentar evolução benigna, pode estar associada a neoplasias da pele. Além disso, as alterações cutâneas características potencialmente ocasionam prejuízo psicossocial, devido aos danos estéticos significativos. Os autores descrevem um caso dessa doença associado a ceratoacantoma e sintetizam os conceitos atuais sobre ela.

Dowling-Degos disease is a rare genodermatosis characterized principally by progressive reticulate hyperpigmentation of the flexures. Although the condition is benign, it may be associated with skin malignancies. Furthermore, the characteristic skin changes may be psychologically and socially detrimental to the patient as a result of the unsightliness of the condition. The present report describes a case in which this disease was associated with a keratoacanthoma, and summarizes current concepts on this skin disorder.

Caso clínico: síndrome de Laugier-Hunziker / Clinical case: Laugier-Hunziker syndrome

Se presentan dos casos clínicos de pacientes con el síndrome de Laugier-Hunziker. Mujer diabética de 57 años con lesiones pigmentadas en mucosa oral. Adolescente de 16 años con pigmentación en lengua, mucosa oral y uñas. Se revisa el tema y se analizan diagnósticos diferenciales.

A digitocompressäo como mecanismo de reduçäo do sangramento em modelo animal / Finger, compression as a mechanism fast stop bleeding following sclerotherapy experimental animals

A esclerose de varizes é uma forma de tratamento largamente difundida para telangiectasia e veias varicosas, porém alguns efeitos indesejáveis podem ocorrer como a hiperpigmentaçao equimose, necrose cutânea e outros. O objetivo deste estudo foi avaliar a digitocompressao como mecanismo de reduçao da equimose e consequentemente da hiperpigmentaçao decorrente do sangramento, em modelo animal. Dois coelhos albinos pesando 2030g e 2300g, respectivamente, foram submetidos à anestesia com cloridrato de cetamina 50mg e cloridrato de 2 (2,6 xilidíno)-5,6 dinidro, 4h-1,3 tiazina. Após a anestesia as hemácias foram marcadas com tecnécio Tc. Em seguida realizou-se 20 punçoes em vasos das orelhas onde em 10 foi feita a digitocompressao e em 10 nao. Observou-se que em todas as punçoes onde houve digitocompressao o sangramento foi insignificante e nao detectado pela presença de radioatividade ao passo que nos locais sem a digitocompressao, em 8 pontos foi significantemente importante. Concluiu-se que a digitocompressao é um mecanismo eficiente e eficaz na reduçao do sangramento em esclerose de veias em modelo animal.