Medical Pearl: Gentian violet to highlight the cornoid lamella in disseminated superficial actinic porokeratosis

Abstract Disseminated superficial actinic porokeratosis is the most common form of porokeratosis in adults, which develops in the third or fourth decade of life, but may also occur later or earlier, more frequently in females, particularly in countries with high sun exposure. Lesions are numerous, uniform, superficial with central atrophy, demarcated by a distinct peripheral ridge, and usually found on sun exposed areas or elsewhere. The disease is inherited as an autosomal dominant condition. We present a 57-year-old retired woman. Her initial skin changes, affecting the face, developed in her thirties, and they have not changed their features since. In the following years, changes developed on the extensor surface of her arms and legs, with more prominent erythema, and then also on other parts of the body, including palms and soles, presenting as dark brown pigmented patches. Her mother had similar changes, and her daughter, who lives abroad, also has them. On examination, the patient presented with facial lesions, patches 2-3 mm wide, with peripheral hyperpigmentation and a pale center. There were multiple, 2-3 mm wide, dark brown lesions on the extremities and trunk. The lesions were either flat or with atrophic center with darker filiform corneal rim. Pathohistolgical examination revealed a "cornoid lamella", which is pathognomonic for the diagnosis of porokeratosis. Auxiliary diagnostic methods were also used - dermoscopy and Gentian violet staining. The patient was advised to avoid sun exposure and to apply photoprotective sunscreens. In conclusion, this is a case report of a disseminated superficial actinic porokeratosis that affected three generations of a family. Our patient developed lesions on palms and soles as well. A review of available world literature shows that this is the second case report of disseminated superficial actinic porokeratosis with palmoplantar involvement.

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Clinical and Dermoscopic Features of Pigmented Disseminated Superficial Actinic Porokeratosis: Case Report and Literature Review

Journal of Cutaneous Medicine and Surgery ◽

10.1177/1203475417733465 ◽

2017 ◽

Vol 22 (2) ◽

pp. 229-231 ◽

Cited By ~ 6

Author(s):

Bahman Sotoodian ◽

Muhammad N. Mahmood ◽

Thomas G. Salopek

Keyword(s):

Case Report ◽

Literature Review ◽

Papillary Dermis ◽

Case Presentation ◽

Skin Tumour ◽

Erythematous Papule ◽

Clonal Proliferation ◽

Disseminated Superficial Actinic Porokeratosis ◽

Cornoid Lamella

Introduction: Porokeratosis is a benign hyperkeratotic skin tumour due to a clonal proliferation of keratinocytes and is characterised by a telltale annular threadlike configuration along the border of a skin-colored to erythematous papule that can expand centrifugally. Case Presentation: We are presenting a clinical and dermoscopic case of pigmented disseminated superficial actinic porokeratosis (DSAP) limited to the upper trunk of a white man with sun-damaged skin. Literature Review and Conclusion: A thorough review of PubMed failed to identify any previous reports on the dermoscopic appearance of pigmented porokeratosis. On dermoscopy, the presence of black dots limited to the periphery of the lesions is due to pigment incontinence and melanophages within the superficial papillary dermis limited to the area below the cornoid lamella. Pigmented DSAP is a unique morphological presentation of porokeratosis, and it is essential to be familiar with its clinical and dermoscopic presentation.

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Coexist of Disseminated Superficial Actinic Porokeratosis and Porokeratosis Ptychotropica with A Novel MVK Gene Mutation: A Case Report and Literature Review

10.21203/rs.3.rs-721945/v1 ◽

2021 ◽

Author(s):

Hongjun Xu ◽

Linfeng Li

Keyword(s):

Gene Mutation ◽

Skin Lesions ◽

Causative Mutation ◽

Case Presentation ◽

Disseminated Superficial Actinic Porokeratosis ◽

Cornoid Lamella ◽

Physical Examinations ◽

Skin Biopsies ◽

Porokeratosis Ptychotropica ◽

Mvk Gene

Abstract Background: Porokeratosis is a rare, acquired or inherited disorder of keratinization. There are numerous clinical types of porokeratosis and they could coexist in one patient and in multiple members of an affected family. However, coexist of disseminated superficial actinic porokeratosis (DSAP) and porokeratosis ptychotropica (Ppt) is rare.Case presentation: A 45-year-old man presented with long-standing skin lesions. Physical examinations found numerous small, brown 2- 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 showed a cornoid lamella in the epidermis. And we identified c.155 G>A mutation of the mevalonate kinase (MVK) gene convertting a serine residue to asparagine (p.Ser52Asn) was the causative mutation for porokeratosis in this family. The 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 by microwave knife for more than 10 times. Conclusion: We report an unusual case of DSAP coexisting with Ppt and identified a novel MVK gene mutation in this patient's family. The microwave knife is an effective and safe therapy for porokeratosis.

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A RARE CASE OF GENITAL POROKERATOSIS

10.36106/gjra/6804078 ◽

2020 ◽

pp. 43-43

Author(s):

Sucheta Pathania

Keyword(s):

Rare Case ◽

Indian Literature ◽

Natal Cleft ◽

Characteristic Finding ◽

Genital Region ◽

Disseminated Superficial Actinic Porokeratosis ◽

Cornoid Lamella ◽

Clinical Variants

Porokeratosis is a keratinization disorder of unknownetiology. It is characterized by well defined annular plaques with central scarring or pigmentation and raised keratotic border. Disseminated superficial actinic porokeratosis, linear porokeratosis, porokeratosispalmaris et plantaris ,punctateporokeratosis are different clinical variants. Keratotic border is microscopically characterized by a column of parakeratotic cells invaginating the epidermis known as cornoid lamella which is a characteristic finding. Porokeratosis localized to the genital region is very rare. These lesions are mostly asymptomatic but there can be occasional pruritus. It mostly occurs on the scrotum followed by penis however adjoining areas like thighs, buttocks, natal cleft can also be involved. There are very few reports in Indian literature of porokeratsis limited to the genital region.

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Penile porokeratosis mimicking annular lichen planus

Our Dermatology Online ◽

10.7241/ourd.20221.30 ◽

2022 ◽

Vol 13 (1) ◽

pp. 109-110

Author(s):

Ngo Binh Trinh ◽

Giang Huong Tran ◽

Hoang Trung Hieu

Keyword(s):

Fungal Infection ◽

Cell Carcinoma ◽

Lichen Planus ◽

Co2 Laser ◽

Granular Layer ◽

Secondary Syphilis ◽

Histological Findings ◽

Disseminated Superficial Actinic Porokeratosis ◽

Topical Retinoids ◽

Cornoid Lamella

Sir, Porokeratosis is a group of cutaneous diseases presented by epidermal keratinization [1]. Herein, we report the case of a patient with porokeratosis who responded well to carbon dioxide (CO2) laser therapy. A 22-year-old Vietnamese male visited our department with an asymptomatic plaque on the penis present for three months. He denied a family history of similar lesions. A cutaneous examination of the penis revealed an annular, well-circumscribed plaque with slightly raised borders with scales (Fig. 1a). Other mucocutaneous lesions were absent. Fungal microscopy, a rapid plasma reagin (RPR) test, and a Treponema pallidum hemagglutination (TPHA) test were negative. Histological findings revealed a hyperkeratotic lesion with a discrete parakeratotic column. There was the presence of a cornoid lamella, which was a parakeratotic column overlying a small vertical zone of dyskeratotic and vacuolated cells within the epidermis (Fig. 2a). There was also a focal loss of the granular layer. A mild lymphocytic infiltrate could be seen around an increased number of capillaries in the underlying dermis (Fig. 2b). CO2 laser removal was performed. There was no recurrence after a twelve-month follow-up (Fig. 1b). However, a hypopigmented scar was seen. Porokeratosis is an uncommon disorder of keratinization with clinical variants, such as classical porokeratosis of Mibelli, disseminated superficial actinic porokeratosis, linear porokeratosis, and porokeratosis palmaris et plantaris disseminata [2]. Porokeratosis involving the genital areas and other adjacent sites is rare [2]. Genital porokeratosis was first described by Helfman in 1985 [3]. More than 69 cases have been reported in the literature [1]. The pathophysiology of genital porokeratosis remains unknown. It has been supposed that porokeratosis is linked to repeated minor frictional trauma. A benign lesion may transform into squamous cell carcinoma or basal cell carcinoma [4]. However, no malignant transformation of genital porokeratosis has been noted in the literature. Genital porokeratosis manifests itself clinically as classic or plaque-type porokeratosis of Mibelli [2]. Histological findings revealed a cornoid lamella with the absence of a granular layer and dyskeratotic cells in the upper spinous zone [2]. Our case may mimic some annular lesions, such as secondary syphilis, fungal infection, and annular lichen planus. Because a fungal examination and syphilis serology were negative, we could exclude fungal infection and annular secondary syphilis. The distinctive histology of porokeratosis such as a cornoid lamella with a decreased granular layer may help to differentiate between porokeratosis and annular lichen planus [4]. Numerous therapeutic methods of treatment exist, including surgical excision, CO2 laser, cryotherapy, topical retinoids, 5% 5-fluorouracil, vitamin D3 analogs, imiquimod cream, and 3% diclofenac gel [2,5].

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