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Abstract Although dermatofibromas are not uncommon benign dermal nodules, palms are rarely involved. Herein, a rare case of palmar dermatofibroma was described, which occurred in a patient with porokeratosis.
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Porokeratosis is a keratinization disorder with unclear etiopathogenesis, varied clinical presentation and characteristic histopathology, and is usually unresponsive to current therapeutic options. Until now, it was considered to be a clonal disorder with immunity, ultra violet radiation and other factors playing important roles in etiopathogenesis. It is now known that abnormalities in the mevalonate pathway are responsible for this clonal keratinization abnormality. New variants of porokeratosis like eruptive bullous, pruriginous, lichen planus like, follicular variants and porokeratoma have been described. While the cornoid lamella is the classical histopathologic feature, dermoscopy and reflectance confocal microscopy make the diagnosis clearer. Development of malignancy in a few variants is a concern. Linear, disseminated superficial actinic and giant lesions are most prone to developing malignancies. Bowen’s disease, squamous cell carcinoma, basal cell carcinoma and even melanoma have been reported in cases of long-standing porokeratosis. Newer modalities of therapy such as photodynamic therapy, ingenol mebutate and HMGCoA inhibitors may play a role in the future
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Objective:To analyze clinical and pathological features of follicular porokeratosis.Methods:Clinical and histopathological data were collected from 8 patients who were diagnosed with follicular porokeratosis in Department of Dermatology, Xijing Hospital, the Fourth Military Medical University from January 2015 to February 2022, and analyzed retrospectively.Results:Among the 8 patients, 6 were males and 2 were females. Their average age at onset was 36 years (range, 6 - 67 years) , and the average duration from onset to diagnosis was 6 years (range, 0.5 - 20 years) . No obvious precipitating factors were found. The patients presented with multiple or solitary, small (diameter < 1 cm) , round, long-standing stable reddish-brown maculopapules or plaques with few or no scales on their surface. Most patients had no subjective symptoms, but for some occasional mild itching sensation. Dermoscopic examination of 4 patients showed that keratotic plugs were all present at the opening of hair follicles. Histopathological manifestations of the 8 skin lesions were similar: roughly normal epidermal thickness or mildly irregular acanthosis, parakeratotic columns only at the infundibulum of hair follicles, and thinner or disappeared granular layer containing some dyskeratotic cells. Of the 8 patients, 6 were treated with surgical excision, topical drugs, or laser cautery, etc. The follow-up results showed that surgical excision could cure the primary skin lesions, but could not prevent new skin lesions, and other treatments were basically ineffective.Conclusions:Follicular porokeratosis, a special type of porokeratosis, mostly occurs on the facial area of middle-aged males. This disease usually progresses slowly, and is histopathologically characterized by unique parakeratotic columns only present at the opening of hair follicles. The diagnosis of follicular porokeratosis should rely on both clinical manifestations and histopathological findings.
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RESUMEN La poroqueratosis es un trastorno de la queratinización, con herencia autosómica dominante en la mayoría de los casos, vinculada posiblemente a la presencia de un clon mutante de queratinocitos en expansión. Tiene diferentes formas clínicas: de Mibelli, actínica superficial diseminada, lineal, puntiforme, palmoplantar y facial atípica. El diagnóstico se confirma con la histología, presentándose típicamente la laminilla cornoide, que no es patognomónica de esta afección. En el tratamiento de esta patología se utilizan desde agentes tópicos como emolientes, 5-fluorouracilo, calcipotriol, imiquimod y retinoides como terapia sistémica. Otras opciones terapéuticas descritas son extirpación quirúrgica, criocirugía y dermoabrasión cuando se trata de formas localizadas o únicas. Se decide reportar el caso de una paciente femenina con poroqueratosis de afección exclusiva del área nasal por su rara frecuencia y escasos reportes sobre esta forma de presentación en la actualidad.
ABSTRACT Porokeratosis is a disorder of keratinization, with AD inheritance in most cases; possibly linked to the presence of a mutant clone of expanding keratinocytes, in addition to the overexpression of the p53 gene that could be related to an oncogenic potential. This disorder has also been associated with factors such as UVR, immunosuppression cases such as HIV +, liver or kidney failure, transplantation of organs that would act as triggers of this disease. It differs from other pathologies due to the presence of a peripheral hyperkeratotic ring which is usually asymptomatic in half of the patients. Clinically it can occur in different ways: porokeratosis de Mibelli, superficial actinic disseminated, linear, punctate, palmoplantar and atypical facial. Diagnosis is confirmed with histology, with the cornoid lamella typically present, which is not pathognomonic of this condition and consists of a parakeratotic keratinocyte column that extends from an invagination of the epidermis through the stratum corneum. The treatment to choose, will depend on the patient´s age; Size and location of the lesions, the aesthetic implications and the general condition of the affected person. They are used from topics such as emollients, 5-fluorouracil, calcipotriol, imiquimod. Retinoids are found as an option for systemic therapy. Another procedure considers surgical removal, cryosurgery and dermabrasion when dealing with localized or unique forms. We report a case of a 44-year-old female patient who presents with compatible dermatosis porokeratosis, affecting only the nasal area.
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Este estudo demonstrou o resultado clínico e histológico do tratamento com laser fracionado não ablativo de paciente com poroqueratose actínica superficial disseminada. A paciente recebeu sete sessões de laser 1340-nm Nd:YAP, com intervalos de quatro a cinco semanas. Biópsias e fotos foram realizadas antes e após o tratamento, o qual foi bem tolerado e trouxe melhora do eritema e da textura das lesões. O seguimento foi de um ano. O exame anatomopatológico após o tratamento revelou pouca modificação da lamela cornoide.
This study demonstrated the clinical and histologic result of the treatment of one disseminated superficial actinic porokeratosis patient with non-ablative fractional laser. The patient was treated with seven sessions of 1340-nm Nd:YAP laser, with 4 or 5 week-intervals. Biopsies and photographs were performed before and after treatment, which was well tolerated and lead to improvement in the erythema and texture of the lesions. There was a 1-year follow-up. Histopathologic examination after treatment revealed little changes in the cornoid lamella.
Subject(s)
Therapeutics , PorokeratosisABSTRACT
Porokeratosis is a rare disorder of epidermal keratinization characterized clinically by annular plaque with thread like hyperkeratotic border with a central groove that expand centrifugally and this border corresponds to coronoid lamellae histologically which are the columns of parakeratosis that overlie an epidermal invagination with loss of granular layer and dyskeratosis of upper spinous keratinocytes. The disorder was erroneously named porokeratosis because the coronoid lamella was initially described as being present over a sweat pore, which is a fixed structure that cannot expand peripherally. Five primary clinical variants have been described: classic porokeratosis of mibelli, disseminated superficial actinic porokeratosis, linear porokeratosis, punctate porokeratosis and porokeratosis palmaris et plantaris disseminate. Porokeratosis ptychotropica one of the rare variants of porokeratosis described by lucker et al which has been added recently in the classification. It is characterized clinically by symmetrical verrucous papules and plaques resembling psoriasis plaque in the gluteal cleft, buttocks and rarely extends to genitalia and histologically by multiple coronoid lamella. We report a case of 43year old female, presented with 10years duration of pruritic raised skin lesion over the left gluteal region. Dermatological examination revealed single well defined erythematous scaly plaque with central atrophy, hyperpigmentation and peripheral thread like elevated border. Histopathological examination revealed multiple coronoid lamella which is the hallmark for porokeratosis ptychotropica, confirmed the diagnosis. The patient was treated with 5-fluorouracil cream. we report this case due to its rarity and the unusual presentation of single plaque of porokeratosis ptychotropica.
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RESUMEN Se realizó la presentación de dos pacientes con poroqueratosis de Mibelli por ser una dermatosis poco frecuente, por lo que debe ser de interés para los profesionales de diferentes disciplinas, bien por relacionarse con el cáncer de piel o con disímiles enfermedades. Los pacientes estudiados fueron: paciente femenino de 62 años de edad, piel blanca y ocupación ama de casa. Fue remitida por presentar piel con fototipo cutáneo II, una lesión en placa de color amarillento, bordes elevados, hiperqueratósicos, centro atrófico de 1 cm de diámetro; localizada en cara ántero-externa de pierna derecha. El otro paciente, de 59 años, sexo masculino, piel blanca, y ocupación pescador. Solicitó los servicios por presentar piel fototipo cutáneo II lesión en placa de color amarillento, bordes elevados, hiperqueratósicos, centro atrófico de 2cm de diámetro, localizada en cara póstero-externa de antebrazo derecho. Se les realizó biopsia de ambas lesiones. El diagnóstico fue la poroqueratosis de Mibelli para la prevención del cáncer de piel. Ambos pacientes presentaron en común fototipo cutáneo II y otras manifestaciones cutáneas inducidas por la exposición solar. La terapéutica utilizada fue la extirpación quirúrgica, que resultó también el proceder diagnóstico Se orientaron medidas para la prevención del cáncer de piel con bloqueadores solares, y protegerse de los rayos solares con ropa adecuaday otros accesorios (AU).
ABSTRACT Two patients with Mibelli´s porokeratosis were presented because it is a few frequent dermatosis that might be of interest for professionals of different disciplines since it is related to skin cancer and to several other diseases. The first studied patients was 62 years old, female, white, and a housewife, remitted due to presenting skin with cutaneous phototype II, a yellowish lesion in plaque, risen, hyperkeratoid borders and atrophic center of 1 cm diameter, located in the anterior-external face of the right leg. The other patient was 59 years old, male, white, and a fisherman. He assisted the service presenting skin with cutaneous phototype II, a yellowish lesion in plaque, risen, hyperkeratoid borders and atrophic center of 2 cm diameter, located in the posterior-external face of the right forearm. A biopsy of both lesions was carried out. The diagnosis was Mibelli´s porokeratosis. Both patients presented a common cutaneous phototype II and skin manifestations induced by the sun exposition. The used therapeutic was the surgical removal, that also was the diagnosis procedure. Preventing skin cancer with sun blockers was oriented and also wearing adequate clothes and other accessories (AU).
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Humans , Male , Middle Aged , Skin Diseases , Skin Neoplasms/prevention & control , Porokeratosis/diagnosis , Porokeratosis/therapy , Photochemotherapy , Biopsy/methods , Carcinoma, Squamous Cell , Health Education , Patient Education as Topic , Risk Factors , Protective Factors , Health PromotionABSTRACT
The rate of malignant transformation in porokeratosis (PK) lesions is approximately 7.5%, and linear PK demonstrates the highest rate of malignancy. An 83-year-old woman presented with a rapidly enlarging mass on her left arm. Variably sized erythematous scaly patches were scattered across the left half of her trunk and arm. Additionally, generalized variably sized brownish annular patches with a hyperkeratotic outer ring were observed on her face, trunk and bilateral arms. A skin biopsy was performed on 3 lesions-a yellowish to erythematous appearing mass, an erythematous scaly patch, and a brownish annular patch. Histopathological evaluation of these 3 lesions revealed squamous cell carcinoma, actinic keratosis, and PK, respectively. The final diagnosis was disseminated superficial PK with linear PK on the left side of the body, and actinic keratosis and squamous cell carcinoma confined to the linear PK lesions. We report a case which represents the progressive and stepwise malignant transformation of PK into squamous cell carcinoma.
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Aged, 80 and over , Female , Humans , Arm , Biopsy , Carcinoma, Squamous Cell , Diagnosis , Keratosis, Actinic , Porokeratosis , SkinABSTRACT
Porokeratosis is a rare epidermal disorder characterized by annular or linear hyperkeratotic plaques with slightly raised thread-like borders, and in most cases, atrophic centers. Disseminated superficial porokeratosis and disseminated superficial actinic porokeratosis (DSAP), which primarily involve sun-exposed areas, are common types of porokeratoses. Histologically, a column of parakeratotic cells, a so-called cornoid lamella, is a hallmark of porokeratosis. Porokeratosis is considered to result from the inability to eliminate an abnormal keratinocyte clone induced by genetic factors and various stimuli, including sunlight, artificial ultraviolet light, viral infections, immunosuppressive conditions (hematologic malignancies, organ transplants, or autoimmune disease), and immunosuppressive therapies. Here, we report a 59-year-old Korean woman with DSAP that developed after narrowband ultraviolet B (NB-UVB) therapy for psoriasis. Our case emphasizes the occurrence of DSAP due to NB-UVB that is able to induce local immunosuppression at the irradiated site; the pathogenesis of DSAP remains unclear.
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Female , Humans , Middle Aged , Clone Cells , Immunosuppression Therapy , Keratinocytes , Phototherapy , Porokeratosis , Psoriasis , Sunlight , Transplants , Ultraviolet Rays , Ultraviolet TherapyABSTRACT
Disseminated superficial actinic porokeratosis (DSAP) is a rare keratinization disorder. It is histopathologically characterized by the presence of coronoid lamellae and clinically by lesions showing central atrophy with elevated borders. Squamous cell carcinoma originating in the classical Mibelli subtype of porokeratosis is well-documented; however, few reports have described squamous cell carcinoma in DSAP lesions. We report the case of a 76-year-old woman with DSAP who developed Bowen's disease, actinic keratosis, and keratoacanthoma on her face.
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Aged , Female , Humans , Atrophy , Bowen's Disease , Carcinoma, Squamous Cell , Keratoacanthoma , Keratosis, Actinic , Porokeratosis , SkinABSTRACT
La poroqueratosis es una dermatosis epidérmica que se caracteriza por lesiones anulares, queratósicas e hiperpigmentadas, únicas o múltiples, de evolución crónica. Se considera como una lesión premaligna con riesgo elevado de producción de carcinoma epidermoide. Se presenta el caso de un paciente de 18 años de edad, que desde los primeros meses de vida presentó lesiones en piel con distribución lineal, diseminadas en cara, tronco y ambas extremidades, además de algunos lechos ungueales, de aspecto hiperqueratósico, con bordes amurallados y centro atrófico de color pardo claro. En el año 2003 se confirmó el diagnóstico histológico de poroqueratosis. El presente artículo expone el tratamiento y evolución de este paciente, afectado por una enfermedad muy poco frecuente, tanto en Cuba como en el resto del mundo.
Porokeratosis is an epidermal dermatosis characterized by single or multiple annular, keratotic and hyperpigmented lesions of chronic evolution. It is considered as a pre-malignant lesion with a high risk of epidermoid carcinoma production. A case of an 18-year-old patient, who, from the first months of life, presented skin lesions with linear distribution, disseminated on the face, trunk and both limbs, as well as some unyielding, hyperkeratotic-looking beds with walled edges and atrophic center of light brown color. In 2003 the histological diagnosis of porokeratosis was confirmed. This article describes the treatment and evolution of this patient, affected by a very rare disease, both in Cuba and worldwide.
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No abstract available.
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Carcinoma, Squamous Cell , Epithelial Cells , PorokeratosisABSTRACT
The clinical data of 7 patients with porokeratosis (PK) were analyzed retrospectively.In 7 PK patients, 4 cases were disseminated superficial actinic porokeratosis (DSAP),1 case was disseminated superficial porokeratosis (DSP),1 case was giant porokeratosis (GP) and 1 case was hypertrophic porokeratosis (HP).The characteristic cutaneous manifestations were annular well-circumscribed keratotic plaques with slightly atrophic center and elevated border.All patients shared a common histological hallmark, the cornoid lamella.Four cases of DSAP patients had family medical history, consistent with autosomal dominant inheritance.DSP, GP and HP patients denied family medical history.Diagnosis of PK should be based on clinical manifestations, family medical history and histopathological examination.
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Objective To investigate the efficacy and safety of ultrapulsed fractional CO2 laser in the treatment of porokeratosis in children.Methods Clinical data were collected from 9 children with porokeratosis in the Department of Dermatology of Beijing Children's Hospital from January 2014 to December 2015,and analyzed retrospectively.These patients were all treated with ultrapulsed fractional CO2 laser in a dynamic superficial stripping (Active FX) mode.The initial energies were 100,200 and 300 mJ/cm2,and the frequencies ranged from 100 to 300 Hz.Before and after the treatment,as well as during the follow-up,confocal laser scanning microscopy was used to evaluate the severity and recovery of skin lesions.Results Of the 9 patients,7 were male,and 2 were female.Their average age was 4.19 ± 3.97 years.After the treatment with ultrapulsed fractional CO2 laser,all of the patients were considered to be cured based on the clinical standard.Some adverse reactions such as erythema,edema and erosion occurred in the 9 patients immediately after the treatment,but all completely regressed within 3-7 days.At 3,6 and 12 months after the treatment,no scars or skin discoloration was observed.The average duration of follow-up was 1 year,and the longest duration of follow-up was 2 year and 4 months.No relapse occurred during the follow-up.Conclusion Ultrapulsed fractional CO2 laser is effective and safe for the treatment of porokeratosis in children.
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Abstract Porokeratosis is a disorder of epidermal keratinization characterized by the presence of annular hyperkeratotic plaques. Its etiopathogenesis is not yet fully understood, but a relationship with immunosuppression has been reported. Dermoscopic examination revealed a classic yellowish-white ring-like structure that resembled “volcanic crater contour” – the so-called cornoid lamella. We describe a case of porokeratosis in a female patient with chronic lymphedema, which was similar to Bowen’s disease due to the many glomerular vessels seen on clinical examination and dermoscopy.
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Humans , Female , Aged , Bowen's Disease/pathology , Bowen's Disease/diagnostic imaging , Porokeratosis/pathology , Porokeratosis/diagnostic imaging , Dermoscopy/methods , Chronic Disease , Diagnosis, Differential , Erythema/pathology , Erythema/diagnostic imaging , Lymphedema/pathology , Lymphedema/diagnostic imagingABSTRACT
Abstract Porokeratosis is a skin disorder clinically characterized by annular plaques with keratotic borders resembling the Great Wall of China and histopathologically by cornoid lamellae. The disease has several clinical variants. Porokeratosis ptychotropica, which has recently become part of these variants, is quite rare and little known. The entity is characterized by verrucous plaques – which may resemble a psoriasis plaque – that affect the regions of the buttocks, most commonly the gluteal cleft, with or without extremity involvement. Itching is often present. We report a rare case of porokeratosis ptychotropica and highlight its unusual manifestation (single plaque), the first case reported in the Brazilian literature.
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Humans , Male , Young Adult , Skin/pathology , Porokeratosis/pathology , Pruritus , Biopsy , Brazil , Buttocks/pathology , Erythema/pathologyABSTRACT
Abstract Porokeratosis represents a group of disorders of epidermal keratinization that are characterized by one or more annular plaques surrounded by a histologically distinctive hyperkeratotic ridge-like border called the cornoid lamella. Many studies showed that organ transplantation and immunosuppression were associated in a significant number of cases. Furthermore, an association with squamous cell carcinoma and basal cell carcinoma has been noted in all variants of porokeratosis. The rarity of this disorder and its atypical clinical presentation – a single lesion on the thumb of an HIV-positive male patient – motivated this report.