Asunto(s)
Poroqueratosis , Humanos , Poroqueratosis/diagnóstico , Estudios Retrospectivos , Biopsia , Diagnóstico DiferencialRESUMEN
Porokeratosis ptychotropica is an uncommon form of porokeratosis, which was initially described in 1995. It is clinically characterized by symmetrical reddish to brown-colored hyperkeratotic, verrucous, or psoriasiform plaques on the perianal and gluteal regions. The lesions tend to integrate and expand centrally, with small peripheral satellite lesions. Early skin biopsy and appropriate diagnosis are essential because malignant change occurs in 7.5% of porokeratotic lesions. Conventional treatment options include topical steroid, retinoid, imiquimod, 5-fluorouracil, isotretinoin, excimer laser, photodynamic therapy, intralesional steroid or bleomycin injection, cryotherapy, carbon dioxide (CO2) laser, and dermatome and excision, but none seem to achieve complete clearance. A 68-year-old woman presented with diffuse hyperkeratotic scaly lichenoid plaques on the buttocks that had persisted for several years. A skin biopsy of the buttocks revealed multiple cornoid lamellae and intense hyperkeratosis. There were some dyskeratotic cells beneath the cornoid lamellae and the granular layer was absent. Porokeratosis ptychotropica was diagnosed based on the characteristic clinical appearance and typical histopathological manifestations. She was treated with a CO2 laser in one session and topical application of urea and imiquimod cream for 1 month. The lesions slightly improved at the 1-month follow-up. We herein present a rare case of porokeratosis ptychotropica.
RESUMEN
BACKGROUND: Porokeratosis is a rare, acquired, or inherited disorder of keratinization. There are numerous clinical types of porokeratosis and they can coexist in one patient and multiple members of an affected family. However, coexistence of disseminated superficial actinic porokeratosis (DSAP) and porokeratosis ptychotropica (Ppt) is rare. CASE SUMMARY: A 45-year-old man presented with long-standing skin lesions. Physical examination identified numerous small, brown 2-mm to 4-mm patches on his face and several hyperkeratotic, verrucous plaques on his trunk and extremities. His father and one of his brothers also had similar lesions for years. Skin biopsies indicated a cornoid lamella in the epidermis. We identified c.155G>A mutation in the mevalonate kinase (MVK) gene, which converted a serine residue to asparagine (p.Ser52Asn) and was causative for porokeratosis in this family. A clinicopathologic diagnosis of DSAP and Ppt with a novel MVK gene mutation was made. The hyperkeratotic plaques on the patient's scrotum were completely removed more than 10 times using a microwave knife. CONCLUSION: An unusual case of DSAP coexisting with Ppt harbored a novel MVK gene mutation also present in the patient's family.
RESUMEN
We report a case of porokeratosis ptychotropica with a rare manifestation. Dermoscopy showed dotted vessels, cerebriform pattern, white scales, and brown and greyish white tracks in the periphery over a red-brown background. A skin biopsy confirmed the diagnosis from the presence of cornoid lamellae.
RESUMEN
Porokeratosis ptychotropica (PP) is a rare variant of porokeratosis characterized by pruritic, symmetrical, red-brown verrucous papules, and plaques most commonly localized within the gluteal fold. Herein, we report the clinical, dermoscopy, and reflectance confocal microscopy (RCM) aspects of a case of PP in a 63-year-old Caucasian woman along with histopathological correlation. Both dermoscopy and RCM were able to recognize the cornoid lamella, the histopathological clue shared by all clinical variants of porokeratosis. These non-invasive techniques may help in the differential diagnosis with other inflammatory/infectious and neoplastic disorders that may clinically resemble PP, but show distinct patterns.
RESUMEN
Porokeratosis ptychotropica is an unusual variant of porokeratosis characterized by papules and plaques located on the buttocks and gluteal cleft and showing multiple coronoid lamellae on histology. In this case report, we present the longitudinal clinical course of porokeratosis ptychotropica in a pediatric patient with individual red-brown hyperkeratotic lesions that enlarged and became confluent prior to surgical intervention. We also discuss the etiology of porokeratosis ptychotropica and review current as well as future treatment options for the disease.
Asunto(s)
Poroqueratosis/diagnóstico , Niño , Progresión de la Enfermedad , Humanos , Masculino , Poroqueratosis/etiología , Poroqueratosis/cirugíaRESUMEN
In a 37-year-old man, diagnosis of verrucous porokeratosis could only be made by histological examination. Previously, the skin lesions on the right buttock had been treated by several dermatologists as psoriasis vulgaris. The clinical picture of both dermatoses was characterized by sharply defined, erythematous papules and plaques. Precise evaluation of the clinical morphology was key for diagnosis. Moreover, in contrast to psoriasis, verrucous porokeratosis is characterized by a high degree of treatment resistance.
Asunto(s)
Nevo Sebáceo de Jadassohn/diagnóstico , Nevo Sebáceo de Jadassohn/patología , Poroqueratosis/diagnóstico , Poroqueratosis/patología , Psoriasis/diagnóstico , Psoriasis/patología , Adulto , Dermoscopía/métodos , Diagnóstico Diferencial , Humanos , Masculino , Piel/patologíaRESUMEN
Porokeratosis ptychotropica represents a rare and under-recognized variant of porokeratosis. There are also alternative descriptions for this disorder in the literature. Since its original description in 1995, additional characteristic features have been showed in case reports published in the literature. These cumulative reports, although still limited in numbers, have helped to further shape and define this entity. A case report and review of published literature on this unusual entity are presented. The specific combination of clinical, morphological and histopathological characteristics that can facilitate recognition of the disorder is discussed. There has been a call for uniformity in terminology and a suggestion for alternative terminology has been made. However, we discuss why the earlier term, porokeratosis ptychotropica, is still preferred.