-
World Journal of Clinical Cases May 2022Porokeratosis is a rare, acquired, or inherited disorder of keratinization. There are numerous clinical types of porokeratosis and they can coexist in one patient and...
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.
PubMed: 35663074
DOI: 10.12998/wjcc.v10.i14.4528 -
Journal of Family Medicine and Primary... Mar 2022Porokeratosis is a keratinization disorder characterized by hyperkeratotic sharply demarcated plaques with central atrophy and histopathologically, by cornoid lamella. A...
Porokeratosis is a keratinization disorder characterized by hyperkeratotic sharply demarcated plaques with central atrophy and histopathologically, by cornoid lamella. A 30-year-old male presented with multiple pruritic dark raised skin lesions over the trunk, face, and upper limbs for past 3 years. Cutaneous examination revealed hyperkeratotic annular plaques with raised margins over face, trunk, and arms. Histopathology revealed marked hyperkeratosis with irregular acanthosis and papillomatosis. Vertical parakeratotic foci and focal hypergranulosis were seen. Hence, a diagnosis of disseminated superficial porokeratosis was made. We present this rare case which may have association with systemic disease, immunosuppression, and malignant transformation.
PubMed: 35495808
DOI: 10.4103/jfmpc.jfmpc_1232_21 -
Clinical Case Reports Apr 2022Porokeratotic adnexal ostial nevus (PAON) is a term encompassing porokeratotic eccrine ostial and dermal duct naevus (PEODDN) and porokeratotic eccrine and hair follicle...
Porokeratotic adnexal ostial nevus (PAON) is a term encompassing porokeratotic eccrine ostial and dermal duct naevus (PEODDN) and porokeratotic eccrine and hair follicle naevus (PEHFN). We present the case of a 7-year-old girl who presented with hyperkeratotic verrucous papules in a blaschkolinear distribution on the sole of her left foot.
PubMed: 35432995
DOI: 10.1002/ccr3.5728 -
Anais Brasileiros de Dermatologia 2022A 34-year-old female was referred to our department, complaining of multiple asymptomatic lesions that appeared two weeks previously. The patient had active nephritis...
A 34-year-old female was referred to our department, complaining of multiple asymptomatic lesions that appeared two weeks previously. The patient had active nephritis with nephrotic syndrome and was treated with immunosuppressive therapies. Physical examination revealed multiple well-circumscribed rounds of flat brownish plaques with slightly elevated borders, some of which were covered by scales. The number of lesions was nine in total. Skin biopsy specimens showed dyskeratotic cells in the thinned epidermis with cornoid lamella, and the absence of a granular cell layer. The development of porokeratosis was considered to be related to immunosuppressive therapy or the activity of nephritis.
Topics: Adult; Epidermis; Exanthema; Female; Humans; Nephritis; Nephrotic Syndrome; Porokeratosis
PubMed: 35321801
DOI: 10.1016/j.abd.2020.08.038 -
International Journal of Trichology 2022Porokeratosis (PK) is an epidermal disorder of keratinization, caused by a clone expansion of a mutated keratinocyte that generates a thin parakeratotic column: the...
Porokeratosis (PK) is an epidermal disorder of keratinization, caused by a clone expansion of a mutated keratinocyte that generates a thin parakeratotic column: the cornoid lamella (CL). Depending on the clinical presentation, we differentiate up to 6 different types of PK. Most frequent locations for the lesions are limbs and trunk, while scalp is rarely affected. The possible origin of the CL in the epidermal appendages has been proposed (ostia of the eccrine glands and infundibulum of the follicle). The first case of PK limited to the scalp with exclusive involvement of the hair follicles is presented in this manuscript. It helps identify the clinical description and especially the trichoscopy as a diagnostic key in follicular PK and also to elucidate if it is an incidental histological finding or a new different clinical variant of PK.
PubMed: 35300103
DOI: 10.4103/ijt.ijt_86_20 -
International Journal of Clinical and... 2022Porokeratosis is a disorder of keratinization with many clinical variants. The histological hallmark feature of porokeratosis is a cornoid lamella. Other accompanying...
Porokeratosis is a disorder of keratinization with many clinical variants. The histological hallmark feature of porokeratosis is a cornoid lamella. Other accompanying features include lichenoid inflammation, atrophy towards the centre of the lesion, dermal cytoid bodies, and adjacent lichenoid changes. Lichenoid keratosis is a benign cutaneous condition, thought to largely represent a degenerating seborrheic keratosis or solar lentigo. The classical histologic appearances are characterized by parakeratosis, epidermal acanthosis, and a dense band of lichenoid lymphocytic infiltrate. Since a lichenoid inflammatory reaction pattern can be seen in porokeratosis it has the potential to be misdiagnosed as a lichenoid keratosis if the cornoid lamella is not identified or missed due to sampling selection. We critically review 104 cases of benign lichenoid keratosis to establish whether any of these cases had features to support a diagnosis of porokeratosis. With 9.6% of cases considered for re-classification, we review clues to reaching this histologic diagnosis.
PubMed: 35265253
DOI: No ID Found -
Indian Journal of Dermatology 2021Porokeratosis ptychotropica (PP) is a rare variant of porokeratosis characterized by pruritic, symmetrical, red-brown verrucous papules, and plaques most commonly...
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 , 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.
PubMed: 35068510
DOI: 10.4103/ijd.ijd_894_20 -
International Journal of Women's... Dec 2021
PubMed: 35028392
DOI: 10.1016/j.ijwd.2021.07.009 -
Indian Journal of Sexually Transmitted... 2021Nonvenereal genital dermatoses form an important category of disorders, and verrucous porokeratosis is a rare and less recognized entity among the same. We present the...
Nonvenereal genital dermatoses form an important category of disorders, and verrucous porokeratosis is a rare and less recognized entity among the same. We present the case of a young adult male with warty growths over scrotum and buttocks for a year. Characteristic cornoid lamellae with typical differentiating features were seen in the histopathology, establishing the diagnosis. This case emphasizes the rare nonvenereal cause for a condition clinically mimicking condyloma acuminata.
PubMed: 34909623
DOI: 10.4103/ijstd.IJSTD_74_19 -
Skin Appendage Disorders Nov 2021The lesions of porokeratosis (PK) lead to skin atrophy and scarring as long as they spread centrifugally. PK affecting the nail unit is seldom described.
BACKGROUND
The lesions of porokeratosis (PK) lead to skin atrophy and scarring as long as they spread centrifugally. PK affecting the nail unit is seldom described.
OBJECTIVE
The aim was to revise the previously reported cases of ungual PK and to present 3 new cases.
METHODS
A PubMed search was performed with the keywords "nail" and "porokeratosis." Previously reported cases as well as 3 new cases are depicted in tables.
RESULTS
Only 11 cases of ungual PK were found; 3 new cases have been added. All patients presented with typical lesions of PK (Mibelli, isolated, segmental, or ostial eccrine types) that happened to affect nails due to nail matrix or nail bed compromise, resulting in mild to severe nail scarring, including irreversible anonychia. The present 3 case series contrast with the previous single case reports.
CONCLUSIONS
PK affecting the nails is exceedingly rare. Changes in nails affected by PK are irreversible, since, as on the skin, this is a chronic scarring process.
PubMed: 34901183
DOI: 10.1159/000516304