Epidermolysis bullosa pruriginosa: A case with a novel mutation and co-existent lichen amyloidosus.

Epidermolysis bullosa pruriginosa is a rare variant of dystrophic epidermolysis bullosa characterized by severely pruritic and cicatricial lesions localized to the extensor extremities. We report a Singaporean Chinese male with epidermolysis bullosa pruriginosa with an underlying novel mutation in the COL7A1 gene. A heterozygous acceptor splice site mutation IVS67‑1G>T probably led to in‑frame skipping of exon 68 (36‑basepairs), resulting in a loss of 12 amino acids. Among his three children, only the youngest son, who had bilateral big toenail thickening, possessed the same mutation. His skin biopsy one decade ago revealed association of focal amyloidosis; a recent skin biopsy showed more established features of lichen amyloidosis. It is debatable whether the cutaneous amyloidosis was a secondary or primary phenomenon. Our report highlights that the diagnosis of epidermolysis bullosa pruriginosa may be obscured when cutaneous amyloidosis is coexistent.

Tattoo reactions-An epidemic on the surge: A report of 3 cases.

Tattooing has been practiced in India since ancient era. It has tremendous religious and spiritual significance. In addition, tattooing for cosmetic purposes has become quite popular in recent times. With this increasing trend, there is also an increased risk of adverse effects. Here, we have described two cases of lichenoid reaction developing to red ink in double- colored tattoos and a case of sarcoidal reaction to green tattoo.

Liquen estriado del adulto: experiencia en un servicio de Dermatología / Adult lichen striatus: experience in dermatology service

Introducción. El liquen estriado es una dermatosis que sigue el trayecto de las líneas de Blaschko, se presenta característicamente en población pediátrica y es rara en pacientes adultos. Objetivo. Describir las características epidemiológicas y clínicas de los pacientes adultos con diagnóstico de liquen estriado diagnosticados en el período 1998-2010 en el Hospital Nacional Daniel Alcides Carrión (HNDAC). Material y Métodos. Estudio descriptivo de tipo serie de casos. Se revisó las historias clínicas de los casos de liquen estriado y se obtuvo datos epidemiológicos (edad, sexo), antecedentes de importancia (comorbilidad) y aspectos clínicos de los pacientes (tiempo de enfermedad, características de las lesiones, síntomas asociados). Los datos obtenidos fueron registrados en un instrumento de recolección de datos. Resultados. Ingresaron al estudio un total de 10 casos, el promedio de edad fue de 27 años (rango 16-48 años), 80% de los casos fueron de sexo femenino. Las lesiones clínicas eran placas formadas por pápulas milimétricas levemente descamativas de color piel, eritematosas o ligeramente hipopigmentada de distribución lineal y que seguían las líneas de Blaschko. Las lesiones se presentaron con mayor frecuencia en miembros inferiores (60%) y tronco (40%). El síntoma más frecuentemente referido porlos pacientes fue prurito (70%). La comorbilidad más frecuente fue atopía que se presentó en 40% de los pacientes. Conclusión. Los pacientes adultos con diagnóstico de liquen estriado del HNDAC presentan características clínicas similares a las descritas en pacientes pediátricos. La atopía constituye la comorbilidad más frecuente.

Back ground. Lichen adult is a dermatosis that follows the Blaschko lines, tipically it present in pediatrics population and is rare in adult patients. Objective. Describe the epidemiological and clinical characteristics with diagnosis of lichen striatus that were diagnosed between1998-2010 at National Hospital Daniel Alcides Carrion (HNDAC). Material and methods. Descriptive study of a case series. Clinical records were reviewed, obtaining epidemiological data (age, genre), background of importance (comorbidity) and clinical aspects of the patients ( time of illness , characteristics of the injuries, associated symptoms) that were recorded in a data collection instrument. Results. Ten cases were enrolled, the age average was 27 years old (range 16-48 years), 80% of cases corresponded to females. Clinical injuries were plates formed by millimeter papules slightly descamatives of skin color, eritematoses or slightly hypopigmentated od linear distribution and that followed the blaschko lines. Injuries were presented with more frequency at the lower limbs (60%) andin trunk (40%). The most frequent symptom was the pruritus (70%).The most frequent comorbidity was the atopy, presents in 40% of the patients. Conclusion. Adult patients with diagnosis of lichen striatus atthe HNDAC present clinical characteristics similar to pediatrics patients. The comorbility more frequent is the atopy.

Lichen amyloidosus: a study of clinical, histopathologic and immunofluorescence findings in 30 cases.

BACKGROUND: Lichen amyloidosus (LA) is a primary localized cutaneous amyloidosis characterized clinically by discrete hyperkeratotic hyperpigmented papules and histologically by deposition of amyloid material in previously normal skin without any evidence of visceral involvement. AIMS AND OBJECTIVES: The aim of this work was to study the etiology, clinical features, histopathology and direct immunofluorescence findings in LA. METHODS: A prospective study of 30 patients with clinical, histological and immunofluorescence findings suggestive of LA was undertaken. After a detailed history and clinical examination, two punch biopsies for histopathology and immunofluorescence were taken. RESULTS: Of the 30 patients, 19 (63.3%) were males and 11 (36.7%) were females with duration of LA ranging from 6-20 months. Pruritus was the presenting symptom in 27 (90%) patients. Shin was involved in 26 (86.7%) followed by arms in three (10%) and back in one (3.3%). Seventeen patients (56%) had used scrubs for more than 2 years. Histopathology, direct immunofluorescence and Congo red staining detected amyloid in all cases. CONCLUSIONS: LA commonly presents over the shins as pruritic discrete hyperpigmented papules. Familial predisposition and friction may have a pathogenic role. Histopathological examination is very useful in the detection of amyloid which may be supplemented with direct immunofluorescence and Congo red staining.