Porokeratosis: A Review of its Pathophysiology, Clinical Manifestations, Diagnosis, and Treatment

Vargas‐Mora, Pablo; Morgado‐Carrasco, Daniel; Fustà‐Novell, Xavier

doi:10.1016/j.adengl.2020.08.005

Cited by 21 publications

(25 citation statements)

References 93 publications

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“…Previous publications, mainly case reports, have not reported frequencies of specific dermoscopic findings in porokeratosis. However, a keratin rim was observed in all previously reported cases of porokeratosis which is in concordance with our cohort in which it was present in over 90% of the cases 2,7,9–26 . The presence of grey‐brown dots or pigmentation along the keratin rim has been reported on several occasions and was detected in 38.5% of lesions in our data set 2,9,10,13,15,21–26 .…”

Section: Discussionsupporting

confidence: 92%

“…However, a keratin rim was observed in all previously reported cases of porokeratosis which is in concordance with our cohort in which it was present in over 90% of the cases 2,7,9–26 . The presence of grey‐brown dots or pigmentation along the keratin rim has been reported on several occasions and was detected in 38.5% of lesions in our data set 2,9,10,13,15,21–26 . The most common previously reported vascular structure in porokeratosis was dotted or glomerular vessels, which was also present in almost 50% of our cases 2,14,16,17,20,21,26 .…”

Section: Discussionsupporting

confidence: 92%

“…Porokeratosis represents a group of relatively uncommon hereditary or acquired skin disorders of unknown aetiology characterized by abnormal keratinization 1 . Some contributing factors for the development of porokeratosis are immunosuppression, ultraviolet radiation and viral infections 1–3 . Nine clinical variants of porokeratosis have been described with varying clinical course, morphology and distribution.…”

Section: Introductionmentioning

confidence: 99%

“…Several differential diagnoses can be considered including actinic keratosis, squamous cell carcinoma in situ , superficial basal cell carcinoma or annular inflammatory conditions (e.g. guttate psoriasis, pityriasis rosea, nummular dermatitis, annular lichen planus, tinea corporis or granuloma annulare) 2,9 …”

Section: Introductionmentioning

confidence: 99%

“…Nine clinical variants of porokeratosis have been described with varying clinical course, morphology and distribution. Nevertheless, the two most common presentations are as follows: porokeratosis of Mibelli, usually presenting with singular lesions on any part of the body, and disseminated superficial actinic porokeratosis (DSAP), with multiple lesions primarily on sun‐exposed skin 2 …”

Section: Introductionmentioning

confidence: 99%

See 4 more Smart Citations

Dermoscopy of porokeratosis: results from a multicentre study of the International Dermoscopy Society

Zaar

Polesie

Navarrete‐Dechent

et al. 2021

Acad Dermatol Venereol

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Background The diagnosis of porokeratosis can be challenging, and knowledge about its dermoscopic features is limited. Objectives To describe the dermoscopic features of porokeratosis of Mibelli and disseminated superficial actinic porokeratosis (DSAP) and the frequency of these features in a larger case series. The interobserver concordance was also assessed. Methods In this retrospective cohort study, members of the International Dermoscopy Society contributed macroscopic and dermoscopic images of histopathologically verified cases of porokeratosis of Mibelli or DSAP. Three observers independently reviewed the collected images to identify the presence of predefined dermoscopic features. Following this, a consensus meeting was held to agree upon which dermoscopic features were present in each lesion. Results In total, 78 clinical and dermoscopic images of porokeratoses were collected. The most common dermoscopic feature was keratin rim, which was present in 74 lesions (92.3%). The most common vascular structures were dotted or glomerular vessels which were present in almost half of the cases (48.7%). Other relatively frequent dermoscopic findings were as follows: non‐peripheral scales (44.9%), grey‐brown dots or pigmentation along the keratin rim (38.5%), and light‐brown pigmentation within the keratin rim (33.3%). Shiny white structures and blood spots or erosions along the keratin rim were findings never before described in porokeratosis and were detected in 16.7% and 17.9% of the lesions, respectively. Dermoscopic findings in porokeratosis of Mibelli and DSAP were similar except for fewer blood spots or erosions along the keratin rim and more light‐brown pigmentation within the keratin rim in DSAP. The interobserver concordance ranged from 0.44 (moderate) to 0.84 (almost perfect). Conclusions The dermoscopic hallmark of porokeratosis is the keratin rim, a finding also allowing for almost perfect interobserver agreement. Pigmentation or erosions along the keratin rim, vascular structures, as well as scales, pigmentation or shiny white structures within the keratin rim are additional dermoscopic clues.

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Section: Discussionsupporting

confidence: 92%

Section: Discussionsupporting

confidence: 92%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 3 more Smart Citations

Dermoscopy of porokeratosis: results from a multicentre study of the International Dermoscopy Society

Zaar

Polesie

Navarrete‐Dechent

et al. 2021

Acad Dermatol Venereol

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Porokeratosis plantaris palmaris et disseminata with pruritic inflammatory changes

Hayashi¹,

Iinuma

Ishida‐Yamamoto

2023

JEADV Clinical Practice

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Porokeratosis plantaris palmaris et disseminata (PPPD) is a rare variant of porokeratosis that begins on the palms and soles and subsequently spreads to the trunk and extremities. We report a case of PPPD with pruritic inflammatory changes in a 48‐year‐old man, presenting with a 3‐year history of pruritic papules that initially appeared on his soles and palms and later spread to his limbs as hyperkeratotic erythematous papules and confluent thick plaques. Skin biopsies revealed cornoid lamellae, along with an inflammatory infiltrate consisting of lymphocytes and histocytes in the upper dermis.

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Approach to the so-called “Invisible Dermatosis”: When Subtle Histopathological Findings Guide Diagnosis

Llamas-Velasco,

Pérez-Muñoz,

Rozas-Muñoz

et al. 2023

The American Journal of Dermatopathology

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Invisible dermatosis is a concept that can be applied either to clinical or histopathological findings. We will focus on the dermatopathological aspect of this invisible dermatosis that can be seen as dermatosis with subtle histopathological findings that are mandatory to known to stablish the diagnosis. With a proper approach facing in depth the different skin layers from stratum corneum to subcutaneous tissue combined with some especial stains, special investigations and mostly a proper clinicopathological correlation, the problem of missing out a diagnosis can be decreased. We will review the general aspects for diagnosis and the peculiar findings of an in-depth review of them because it is important to note that minor changes on a skin biopsy do not mean it is disease free. We will review classic clues, we will add some new useful ones, and we will also provide a guide on the special stains helpful, such as periodic acid–Schiff when facing fungi, orcein-Giemsa and van Gieson when altered elastic fibers are suspected, or Pearl and Masson Fontana when an altered skin pigmentation is suspected.

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Porokeratosis: A Review of its Pathophysiology, Clinical Manifestations, Diagnosis, and Treatment

Cited by 21 publications

References 93 publications

Dermoscopy of porokeratosis: results from a multicentre study of the International Dermoscopy Society

Dermoscopy of porokeratosis: results from a multicentre study of the International Dermoscopy Society

Porokeratosis plantaris palmaris et disseminata with pruritic inflammatory changes

Approach to the so-called “Invisible Dermatosis”: When Subtle Histopathological Findings Guide Diagnosis

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