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Cureus May 2023Linear porokeratosis (LP) is an epidermal keratinization disorder manifesting in the form of annular plaques with an atrophic center and hyperkeratotic margins. Although...
Linear porokeratosis (LP) is an epidermal keratinization disorder manifesting in the form of annular plaques with an atrophic center and hyperkeratotic margins. Although rare, LP carries a significant risk of skin cancer. Histological examination usually reveals the cornoid lamella, a parakeratosis column visualized in the outer layer of the epidermis. First-line treatment of LP is retinoids. However, the effects of combination therapy of isotretinoin and topical statins on LP are not well-understood. Herein, we attempted treatment with both isotretinoin and 2% cholesterol/atorvastatin ointment, with considerable improvement observed using the former but not the latter. These findings suggest that 2% topical cholesterol/atorvastatin treatment may not carry any additional benefits, even if used alongside retinoids. Further studies are needed to assess the potential effects of statins on LP.
PubMed: 37303383
DOI: 10.7759/cureus.38873 -
Clinical, Cosmetic and Investigational... 2023Porokeratosis ptychotropica (PPt) is a rare type of porokeratosis (PK) characterized by pruritic, reddish-brownish verrucous papules, and plaques usually around genital...
Porokeratosis ptychotropica (PPt) is a rare type of porokeratosis (PK) characterized by pruritic, reddish-brownish verrucous papules, and plaques usually around genital area or buttocks. Here, a case of a 70-year-old woman who was diagnosed as PPt was reported. The patient suffered from severe pruritic papules and plaques in the buttock region and pubis for 4 years. The skin lesions were giant, well-defined brown plaques with many satellite papules scattered around. Both clinical manifestations and histopathological features supported the diagnosis of PPt. In review of the identified mutation was found in patients with disseminated superficial actinic porokeratosis (DSAP) combined with PPt, while its unclear in PPt. To investigate the hypothesis that the variant reported in the present case report may played as an independent "likely pathogenic factor" of PPt. Consequently, a de novo missense pathogenic mutation in the MVK gene was identified in this case. Unexpectedly, it is a first report of a novel MVK mutation in sporadic PPt. This rare case suggested an isogenetic background between PPt and DSAP, which may help to explore the underlying pathogenesis of PPt.
PubMed: 37250911
DOI: 10.2147/CCID.S408016 -
Dermatopathology (Basel, Switzerland) May 2023Spiny keratoderma (SK) was first described by Brown in 1871 and is characterized by numerous 1-2 mm spines of keratin on the palms and soles, usually sparing the dorsal...
Spiny keratoderma (SK) was first described by Brown in 1871 and is characterized by numerous 1-2 mm spines of keratin on the palms and soles, usually sparing the dorsal surfaces, or disseminated over the trunk. Histologically, the "spine" represents a column of hyperkeratosis. Several different forms are known, including familial, sporadic, post-inflammatory and paraneoplastic. Although an association of SK with melanoma has been reported, the significance of such co-occurrence remains unclear due to the limited number of cases. To increase the body of knowledge and shed further light on this rare condition, we present a case of SK in a patient with a recent history of melanoma in situ.
PubMed: 37218903
DOI: 10.3390/dermatopathology10020021 -
Actas Dermo-sifiliograficas Apr 2024
Topics: Humans; Porokeratosis; Biopsy
PubMed: 37150245
DOI: 10.1016/j.ad.2022.07.037 -
JAAD Case Reports May 2023
PubMed: 37078016
DOI: 10.1016/j.jdcr.2023.02.009 -
The Journal of Clinical and Aesthetic... Apr 2023No known studies have attempted to describe the pathophysiological relationship between patients who develop both porokeratosis and hidradenitis suppurativa (HS). The...
OBJECTIVE
No known studies have attempted to describe the pathophysiological relationship between patients who develop both porokeratosis and hidradenitis suppurativa (HS). The purpose of this report is to present possible immunological mechanisms that predispose patients to developing both porokeratosis and HS.
METHODS
In this case series, patients were identified during routine clinical encounters and data was extracted from the electronic medical record from October 2010 until April 2021. This study is a single center case series including patients from the department of dermatology at the UNC School of Medicine in Chapel Hill, North Carolina. Patients were selected via digital chart review if they had simultaneous diagnoses of disseminated porokeratosis and HS. Two eligible patients were identified as actively receiving care. One patient is a Black female and the other a White male. No primary study outcomes were planned. This investigation utilized chart review to identify disease time course, which was subsequently used to elucidate study outcomes.
RESULTS
Patient A is a 54-year-old Black female and Patient B is a 65-year-old White male. Both patients developed porokeratosis after multiple years of living with HS. Immunosuppression with adalimumab, corticosteroids, or other medications did not clearly precede porokeratosis development in either patient.
LIMITATIONS
Limitations include that this study was conducted at a single center and prevalence of patients with concomitance of both conditions is low.
CONCLUSION
In patients who demonstrate simultaneous HS and porokeratosis, activation of the innate immune system and associated IL-1 production may lead to autoinflammation and a phenotype of hyperkeratinization. Mutations in genes such as mevalonate kinase may predispose subjects to the development of porokeratoses and HS.
PubMed: 37077926
DOI: No ID Found -
Indian Journal of Dermatology 2022We report a case of porokeratosis ptychotropica with a rare manifestation. Dermoscopy showed dotted vessels, cerebriform pattern, white scales, and brown and greyish...
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.
PubMed: 36998821
DOI: 10.4103/ijd.ijd_1145_20 -
JAMA Dermatology May 2023Disseminated superficial actinic porokeratosis (DSAP) is an inherited or sporadic disorder of keratinization associated with germline variations. There is no effective... (Randomized Controlled Trial)
Randomized Controlled Trial
Safety and Efficacy of Topical Lovastatin Plus Cholesterol Cream vs Topical Lovastatin Cream Alone for the Treatment of Disseminated Superficial Actinic Porokeratosis: A Randomized Clinical Trial.
IMPORTANCE
Disseminated superficial actinic porokeratosis (DSAP) is an inherited or sporadic disorder of keratinization associated with germline variations. There is no effective standard of care therapy for DSAP, but treatment with topical lovastatin combined with cholesterol cream has shown promise.
OBJECTIVES
To evaluate and compare the safety and efficacy of topical lovastatin 2% plus cholesterol 2% cream (lovastatin-cholesterol) and topical lovastatin 2% cream (lovastatin) alone in adults diagnosed with DSAP.
DESIGN, SETTING, AND PARTICIPANTS
This patient- and assessor-blinded, randomized clinical trial was conducted at the Medical University of South Carolina between August 3, 2020, and April 28, 2021. Nonpregnant adults with a previous clinical or histological diagnosis of DSAP were eligible. Data were blindly analyzed after study completion.
INTERVENTIONS
Participants were randomized to once- or twice-daily application of either lovastatin-cholesterol cream (n = 17) or lovastatin cream (n = 14) to symptomatic regions for 12 weeks.
MAIN OUTCOMES AND MEASURES
The primary efficacy measure was the effect of the treatment on DSAP at the end of treatment (12 weeks) as measured by the DSAP General Assessment Severity Index (DSAP-GASI; scored from 0-4, with 0 indicating clear and 4 indicating severe). Treatment efficacy was based on investigator-standardized photographs provided by the participants because of the need for evaluation via telehealth during the COVID-19 pandemic. Secondary efficacy measures included patient-reported outcomes, application frequency, and adverse events (AEs).
RESULTS
Of the 87 participants screened, 32 were enrolled. One participant randomized to receive lovastatin-cholesterol did not receive the intervention, leaving 17 participants (mean [range] age, 59.2 [40-83] years; 13 females [76.5%]; all White) allocated to receive lovastatin-cholesterol treatment and 14 participants (13 female [92.9%]; mean (range) age, 53.7 [33-71] years; all White) to receive lovastatin treatment. Twelve participants in each treatment group qualified for the analysis. Disease severity decreased from week 1 to week 12 by 50.0% (from 3.08 [95% CI, 2.57-3.60] to 1.54 (95% CI, 1.04-2.05] points on the DSAP-GASI; P < .001) in the lovastatin-cholesterol group and 51.4% (from 2.92 [95% CI, 2.40-3.43] to 1.50 [95% CI, 0.99-2.01] points; P < .001) in the lovastatin group. There was no significant difference between the treatment groups according to application frequency at the end of 12 weeks. Adverse events reported included myalgia (n = 2), elevation in the creatine kinase level (n = 1), application discomfort (n = 4), and rash (n = 1). No serious AEs occurred, and all participants with an AE were able to complete the study.
CONCLUSIONS AND RELEVANCE
This randomized clinical trial found improvements in DSAP severity in both treatment groups, without serious AEs, indicating a limited benefit with the addition of cholesterol. These results suggest that lovastatin cream may be a new primary treatment option for patients diagnosed with DSAP.
TRIAL REGISTRATION
ClinicalTrials.gov Identifier: NCT04359823.
Topics: Adult; Humans; Female; Middle Aged; Lovastatin; Porokeratosis; Pandemics; COVID-19; Treatment Outcome; Emollients; Cholesterol
PubMed: 36947042
DOI: 10.1001/jamadermatol.2023.0205 -
Actas Dermo-sifiliograficas Jun 2023
Topics: Humans; Porokeratosis; Hydroxymethylglutaryl-CoA Reductase Inhibitors; Imiquimod; Administration, Cutaneous; Cholesterol
PubMed: 36925100
DOI: 10.1016/j.ad.2022.06.025