Coexistence of porokeratosis of Mibelli and disseminated superficial actinic porokeratosis

( Eun Sun Kwon ),( Byoung Yong Koh ),( Ki Bum Myung ),( Seung Hyun Cheong ) 대한피부과학회 2015 대한피부과학회 학술발표대회집 Vol.67 No.1

Porokeratosis is an uncommon disorder of keratinization. It is characterized histopathologically by a parakeratotic column extending the stratum corneum, known as the cornoid lammella. Porokeratosis has been classified six clinical variants. Although some reports have been described the coexistence of different porokeratotic variants, the coexistence of porokeratosis of Mibelli(PM) and disseminated superficial actinic porokeratosis(DSAP) is rare. A 74-year-old man presented multiple pruritic brown annular macules and papules on face, neck, arms and brown plaques with hyperkeratotic border on lower legs. The large plaques on lower legs were noticed at childhood and many members of his family also had similar skin lesions. Then, Multiple brown annular macules and papules with mild itching on face, neck and both arms occured 4 years ago and the counts gradually increased. Both biopsy specimens taken from lower leg and face revealed the characteristic histologic features of porokeratosis. Herein, we report a case of coexistence of porokeratosis of Mibelli and disseminated superficial actinic porokeratosis.

Linear porokeratosis superimposed on disseminated superficial porokeratosis

( Seuk Hoon Moon ),( Ki Min Sohn ),( Hei Sung Kim ),( Sang Hyun Cho ),( Jeong Deuk Lee ) 대한피부과학회 2015 대한피부과학회 학술발표대회집 Vol.67 No.1

Porokeratosis is a group of genetic disease characterized by a clonal proliferation of keratinocytes. To date, 6 different types of porokeratosis have been identified. Disseminated superficial actinic porokeratosis (DSAP) and linear porokeratosis (LP) are included in this group. A 16-year-old female presented with pruritic, multiple, linear and disseminated, annular, brown macules with a peripheral rim arranged on the right lateral side of the upper and lower extremities for 10 years. Histologic findings revealed hyperkeratosis, acanthosis and cornoid lamellae lacking a granular layer, which is suggestive of DSAP. Clinically, some lesions were scattered, and others were arranged on a linear pattern along Blaschko``s lines. With the clinical and histological findings, a diagnosis of linear porokeratosis superimposed on disseminated superficial porokeratosis was made. We attempted treatment with topical tretinoin and Q-switched Nd:YAG laser, but the patient is currently lost for follow up. The coexistence of different forms of porokeratosis in a single person is uncommon, and around 28 cases have been reported so far. Few reports have been made on the association between LP and DSAP. Although several surgical or medical treatments have been tried, none of them have shown consistency and long-term efficacy. Herein, we report an interesting case of linear porokeratosis superimposed on disseminated superficial porokeratosis.

한공각화중에서 p53 단백질과 Ki-67 항원의 발현

김성준(Sung Jun Kim),오창근(Chang Keun Oh),김문범(Moon Bum Kim),장호선(Ho Sun Jang),권경술(Kyung Sool Kwon) 대한피부과학회 2001 大韓皮膚科學會誌 Vol.39 No.11

N/A Background: Porokeratosis is a rare group of disorders of epidermal keratinization which is histologically characterized by the presence of cornoid lamella. The malignant potential of porokeratotic lesions is well recognized. Recently, frequent p53 overexpression has been reported and might be related to the carcinogenic potential of porokeratosis. Objective : To compare previous foreign results of p53 overexpression in porokeratosis with Korean cases, we studied 24 Korean cases of porokeratosis(7 of Porokeratosis of Mibelli, 14 of DSAP, 2 of Linear Porokeratosis, and 1 of PPPD). Methods : Immunohistochemical staining was done on the paraffin sections using a labelled streptavidin-biotin-peroxidase complex method with primary antibodies against p53 protein and Ki-67. Results . The epidermis central to cornoid lamellae was positive for p53 protein in 15 of the 24 cases. The epidermis beneath the cornoid lamellae was positive in 3 of the central positive 15 cases and the peripheral epidermis was positive in 1 of the central positive 15 cases. Staining for Ki-67 antigen was increased above background levels in 9 of 24 specimens. No correlation was detected between p53 protein expression and Ki-67 antigen expression. Conclusion : The p53 overexpression was observed in Korean cases of porokeratosis but, the expression rate of p53 in Korean cases of porokeratosis was relative less than previous foreign reports.

Unusual changes of disseminated superficial porokeratosis: actinic keratosis and focal acantholytic dyskeratosis

( Wooseok Jeong ),( Woojung Jin ),( Seunghyun Moon ),( Taekgeun Lee ),( Taegwang Kwon ),( Sookkyung Lee ) 대한피부과학회 2016 대한피부과학회 학술발표대회집 Vol.68 No.1

Porokeratosis is a disorder of epidermal keratinization manifested clinically by a raised border and histologically by a cornoid lamella. Although porokeratosis has been regarded as a benign entity, malignant transformation has been reported to occur in 7.5 to 11 percent of patients with porokeratosis. Focal acantholytic dyskeratosis(FAD) has a distinctive histologic pattern characterized by suprabasal clefts, acantholytic and dyskeratotic cells which have been observed as an incidental finding in a variety of skin lesions. A 69-year-old man who had underlying disseminated superficial porokeratosis(DSP) presented with a 1.5 X 1.5 cm sized irregular shaped red to brown colored plaque with oozing on his left upper arm. Histologic feature revealed cornoid lamella and architectural disarray, atypical keratinocytes with cellular and nuclear polymorphism which was compatible with actinic keratosis. A 60-year-old woman who had underlying DSP presented with a 1.0 X 0.8 cm sized yellow crusted erythematous plaque on her right lower back. Histologic feature revealed cornoid lamella and extensive acantholysis with suprabasal clefting which was compatible with FAD. There have not been any prior reports of FAD associated with porokeratosis. Herein, we report two unusual changes, actinic keratosis and FAD arising from DSP and we suggest careful observation should be warranted for patient with porokeratosis.

A Case of Linear Porokeratosis Superimposed on Disseminated Superficial Actinic Porokeratosis

( Seok Hoon Moon ),( Hei Sung Kim ),( Jeong Deuk Lee ),( Sang Hyun Cho ) 대한피부과학회 2016 대한피부과학회지 Vol.54 No.10

Porokeratosis is a group of genetic diseases characterized by clonal proliferation of keratinocytes. We report a 16-year-old female who presented with both linear porokeratosis and disseminated superficial actinic porokeratosis. The unusual coexistence of these two types of porokeratosis is an example of a type 2 segmental manifestation of an autosomal dominant skin disorder. (Korean J Dermatol 2016;54(10):819∼821)

[P230] Disseminated superficial actinic porokeratosis in a patient with psoriasis after long-term narrowband ultraviolet B phototherapy

( Chang Yoon Sim ),( Seung Min Lee ),( Hyun Ju Kim ),( Kyu Uang Whang ),( Young Lip Park ),( Sung Yul Lee ) 대한피부과학회 2017 대한피부과학회 학술발표대회집 Vol.69 No.1

Porokeratosis is a rare epidermal disorder characterized by annular or linear hyperkeratotic plaques with slightly raised theread-like borders, and in most cases atrophic centers. Disseminated superficial porokeratosis (DSP) and disseminated superficial actinic porokeratosis (DSAP), which especially involve sun-exposed areas, are common types of porokeratoses. Histologically, a column of parakeratotic cells-the so called ‘‘cornoid lamella’’ is a hallmark for porokeratosis. Porokeratosis is considered to occur as a result of being unable to eliminate an abnormal clone of keratinocytes induced by genetic factors and stimuli including sunlight and artificial ultraviolet light, viral infections, immunosuppressive conditions (hematologic malignancies, organ transplant, or autoimmune disease), and immunosuppressant therapies. Here, we report a case on an 59-year-old Korean woman with DSAP after narrowband ultraviolet B (NB-UVB) therapy for psoriasis. Our case emphasizes the occurrence of DSAP due to NB-UVB that is able to induce local immunosuppression on the irradiated site, although the pathogenesis of DSAP is unclear.

Linear porokeratosis on the sole mimicking verruca vulgaris

( Do Hun Kim ),( Gwang Hoon Kim ),( Tae Han Kim ),( Nam Hee Sung ),( Hyoseung Shin ),( Ai Young Lee ),( Seung Ho Lee ) 대한피부과학회 2015 대한피부과학회 학술발표대회집 Vol.67 No.1

Porokeratosis is a clonal disorder of keratinization characterized by hyperkeratotic papules or plaques surrounded by a ridge-like elevated border called the cornoid lamella. Porokeratosis is divided into several clinical variants. Linear porokeratosis is an uncommon variant that usually presents in infancy or early childhood. Lesions of linear porokeratosis arise as reddish brown patches or linear keratotic papules and plaques typically distributed along Blaschko``s lines. Usually, lesions of linear porokeratosis are unilaterally confined to one extremity of distal portion. A 40-year-old female presented with linear hyperkeratotic macules, papules and plaques on the right sole and ankle since young adult. The skin lesion had been diagnosed as verruca vulgaris and some parts of the lesion had been treated with carbondioxide laser in other hospital before. The treated lesion improved slightly with scar formation. Histopathologic examination showed marked acanthosis with parakeratotic column, the cornoid lamella. These clinical and histopathologic findings were consistent with linear porokeratosis.

Disseminated Superficial Actinic Porokeratosis in a Patient with Psoriasis, after Long-Term Narrowband Ultraviolet B Phototherapy

( Chang Yoon Sim ),( Ji Yeoun Shin ),( Sung Yul Lee ),( Young Lip Park ) 대한피부과학회 2018 Annals of Dermatology Vol.30 No.2

Porokeratosis is a rare epidermal disorder characterized by annular or linear hyperkeratotic plaques with slightly raised thread-like borders, and in most cases, atrophic centers. Disseminated superficial porokeratosis and disseminated superficial actinic porokeratosis (DSAP), which primarily involve sun-exposed areas, are common types of porokeratoses. Histologically, a column of parakeratotic cells, a so-called cornoid lamella, is a hallmark of porokeratosis. Porokeratosis is considered to result from the inability to eliminate an abnormal keratinocyte clone induced by genetic factors and various stimuli, including sunlight, artificial ultraviolet light, viral infections, immunosuppressive conditions (hematologic malignancies, organ transplants, or autoimmune disease), and immunosuppressive therapies. Here, we report a 59-year-old Korean woman with DSAP that developed after narrowband ultraviolet B (NB-UVB) therapy for psoriasis. Our case emphasizes the occurrence of DSAP due to NB-UVB that is able to induce local immunosuppression at the irradiated site; the pathogenesis of DSAP remains unclear. (Ann Dermatol 30(2) 211∼213, 2018)

한공각화증과 광선각화증에서 세포사멸과 p53, bcl-2, PCNA 및 iNOS 발현에 관한 연구 (제1보)

명기범(Ki Bum Myung) 대한피부과학회 2000 대한피부과학회지 Vol.38 No.2

N/A Background:Porokeratosis is characterized by cornoid lamellae histologically and evolve cutaneous malignant tumors in about 7% of the patients. Actinic keratosis is the most common premalignant lesion and progressed into squamous cell carcinoma in 20% of the patients. It has been reported that the expression of p53 increased within or beneath the cornoid lamellae of porokeratosis and actinic keratosis. Objective:The purpose of this study was to understand the role of apoptosis in pathogenesis and progression into malignant tumors and to investigate whether a positive correlation occurs between apoptosis index and expression of p53, bcl-2, PCNA and iNOS in porokeratosis and actinic keratosis. Methods:TUNEL staining and immunohistochemical staining with p53, bcl-2, PCNA and iNOS antibody were done in paraffin-embedded tissue sections of 10 cases of porokeratosis and 12 cases of actinic keratosis. Results:The results are summarized as follow: 1. The mean of apoptosis index(%) was 27.3±7.82 in porokeratosis, 36.3±10.32 in cornoid lamellae of porokeratosis. 2. The mean of apoptosis index(%) of actinic keratosis was 41.5±8.98 and statistically significant higher than that of porokeratosis(p=0.002). (Korean J Dermatol 2000;38(2):207~212)

염증성 표재성 파종상 한공각화증

심현수 ( Hyun Soo Shim ),정소영 ( So Young Jung ),이현재 ( Hyun Jae Lee ),최준희 ( Joon Hee Choi ),서종근 ( Jong Keun Seo ),이드보라 ( Deborah Lee ),성호석 ( Ho Suk Sung ) 대한피부과학회 2010 대한피부과학회지 Vol.48 No.7

Inflammatory disseminated superficial porokeratosis is an unusual pruritic variant of disseminated superficial porokeratosis, and this was first described by Kanzaki in 1992 as eruptive pruritic papular porokeratosis. Since the first report, 8 cases of inflammatory disseminated superficial porokeratosis have been reported in English language dermatologic literature and only one case has been reported in the Korean dermatologic literature. The condition is clinically characterized by an acute pruritic exacerbation of disseminated superficial porokeratosis, which had been asymptomatic for several years. Usually the skin lesions show improvement after several months, leaving brownish atrophic lesions. Histopathologic examination reveals the cornoid lamella with an infiltration of eosinophils and lymphocytes in the upper dermis. Herein, we report on an 81-year-old Korean man with inflammatory disseminated superficial porokeratosis, and this was clinically and histopathologically diagnosed. (Korean J Dermatol 2010;48(7):590∼593)