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Dermatology Practical & Conceptual Apr 2024
PubMed: 38810068
DOI: 10.5826/dpc.1402a111 -
Cureus Apr 2024This case report presents a rare instance of Eruptive Pruritic Papular Porokeratosis (EPPP) in a 71-year-old Chinese male, emerging on atypical sites (face, scalp, and...
This case report presents a rare instance of Eruptive Pruritic Papular Porokeratosis (EPPP) in a 71-year-old Chinese male, emerging on atypical sites (face, scalp, and ears) following a COVID-19 infection, and explores the potential link between viral infections and EPPP onset. The patient's lesions, characterized by annular brown patches with hyperkeratotic ridges, showed significant improvement following treatment with Baricitinib and Acitretin. This case underscores the need for awareness of unusual presentations of EPPP and suggests the potential efficacy of Janus Kinase (JAK) inhibitors in treatment, prompting further research into the pathophysiological connections between EPPP and viral infections. Adherence to the SCARE 2023 guidelines ensures a comprehensive and transparent case presentation.
PubMed: 38689674
DOI: 10.7759/cureus.57650 -
Indian Journal of Dermatology,... Apr 2024
PubMed: 38594979
DOI: 10.25259/IJDVL_880_2023 -
Cureus Feb 2024Porokeratosis encompasses a diverse group of dermatoses, both acquired and genetic, marked by a keratinization disorder. Porokeratosis of Mibelli (PKM) presents as...
Porokeratosis encompasses a diverse group of dermatoses, both acquired and genetic, marked by a keratinization disorder. Porokeratosis of Mibelli (PKM) presents as solitary plaques or multiple papules/macules with central atrophy and raised hyperkeratotic borders. Here, we present a case of giant porokeratosis (GPK), a rare form often considered a morphological variant of PKM, with unique clinical and histopathological aspects. Our case involves a 29-year-old patient with a 15 × 10 cm irregular plaque on the dorsal aspect of the right hand. The patient was previously prescribed various topical treatments (retinoids, calcineurin inhibitors, and combinations of corticosteroids with vitamin D3 analogs) and systemic retinoids without improvement before presenting to our department. Due to the high risk of neoplastic transformation and the unavailability of imiquimod, the patient was recommended topical 5-fluorouracil treatment. The trajectory of the lesion under treatment revealed a favorable evolution, and the patient was subjected to regular monitoring every three months to assess the ongoing progress. Recognizing GPK as a high-risk variant is crucial for dermatologists, and it requires a personalized approach. Regular monitoring is advised to detect potential malignant transformations promptly. Future research holds promise for diagnostic advancements, refined treatment modalities, and a deeper understanding of the molecular mechanisms underlying malignancy in porokeratosis.
PubMed: 38558715
DOI: 10.7759/cureus.55155 -
Nagoya Journal of Medical Science Feb 2024Whole-exome and whole-genome sequencing have become widespread in approximately the last 15 years, and the predisposing factors and pathomechanisms of inflammatory... (Review)
Review
Whole-exome and whole-genome sequencing have become widespread in approximately the last 15 years, and the predisposing factors and pathomechanisms of inflammatory keratinization diseases, which have been unknown for a long time, have gradually been revealed. Hence, various inflammatory keratinization diseases are recognized to cause innate immunity hyperactivation. Therefore, we have been advocating for the clinical entity, "autoinflammatory keratinization diseases (AiKDs)" since 2017. AiKDs are inflammatory keratinization diseases caused by autoinflammatory-related pathomechanisms in the skin. The aberrant activation of innate immunity and the resultant autoinflammation in the epidermis and the superficial dermis in AiKDs cause hyperkeratosis in the epidermis. Our initially proposed concept of AiKDs included generalized pustular psoriasis and related conditions, pityriasis rubra pilaris type V, and familial keratosis lichenoides chronica. Since then, the number of diseases known to be AiKDs has increased as previously unknown disease-causing factors and pathogenetic mechanisms of inflammatory keratinization diseases have been clarified one by one. To date, porokeratosis, hidradenitis suppurative, keratosis linearis with ichthyosis congenita and sclerosing keratoderma (KLICK) syndrome, and AiKDs associated with epidermal growth factor receptor (EGFR) deficiency or with hepatitis and autism have been recognized as AiKDs. The concept of AiKDs is considered extremely useful in our precise understanding of the pathogeneses behind inflammatory keratinization diseases and our appropriate treatment method selection. The number of AiKDs is expected to grow with the clarification of the pathomechanisms of further inflammatory keratinization diseases.
Topics: Humans; Keratosis; Skin; Skin Neoplasms; Syndrome
PubMed: 38505726
DOI: 10.18999/nagjms.86.1.1 -
Indian Journal of Dermatology 2023Psoriasis and porokeratosis are two common skin diseases. Nevertheless, the occurrence of comorbidity is rare. To the best of our knowledge, these kinds of clinical...
Psoriasis and porokeratosis are two common skin diseases. Nevertheless, the occurrence of comorbidity is rare. To the best of our knowledge, these kinds of clinical histopathologic features of comorbidity were rarely reported previously. Here, we describe a case of a 75-year-old patient who presented with diffused porokeratosis in association with psoriasis.
PubMed: 38371558
DOI: 10.4103/ijd.ijd_484_22 -
Dermatology Practical & Conceptual Jan 2024
PubMed: 38364379
DOI: 10.5826/dpc.1401a17 -
Actas Dermo-sifiliograficas Apr 2024
Topics: Humans; Porokeratosis; Biopsy
PubMed: 38325540
DOI: 10.1016/j.ad.2022.07.052 -
Cureus Jan 2024Disseminated superficial porokeratosis is a rare dermatological disorder characterized by annular keratotic lesions, presenting diagnostic challenges due to its variable...
Disseminated superficial porokeratosis is a rare dermatological disorder characterized by annular keratotic lesions, presenting diagnostic challenges due to its variable clinical manifestations. The pathogenesis involves genetic predisposition and environmental factors, with mutations in the mevalonate pathway implicated. Despite its benign nature, this condition significantly impacts patients' quality of life, necessitating accurate diagnosis and effective therapeutic strategies. We present the case of a 45-year-old female with a three-year history of annular plaques on sun-exposed areas progressing to involve multiple body regions. The characteristic histopathological finding of coronoid lamellae confirmed the diagnosis of disseminated superficial porokeratosis. Treatment involved a multimodal approach, including topical corticosteroids, calcineurin inhibitors, and systemic retinoids, resulting in satisfactory clinical outcomes. Long-term follow-up emphasized the need for ongoing disease monitoring and patient education regarding sun protection. The presented case underscores the importance of recognizing characteristic histopathological features for accurate diagnosis and highlights the significance of long-term follow-up, disease monitoring, and patient education to optimize outcomes and enhance overall quality of life.
PubMed: 38318598
DOI: 10.7759/cureus.51736