No abstract available.
Porokeratosis

No abstract available.
Photochemotherapy ; Porokeratosis

No abstract available.
Porokeratosis*

Disseminated superficial actinic porokeratosis (DSAP) is a rare keratinization disorder. It is histopathologically characterized by the presence of coronoid lamellae and clinically by lesions showing central atrophy with elevated borders. Squamous cell carcinoma originating in the classical Mibelli subtype of porokeratosis is well-documented; however, few reports have described squamous cell carcinoma in DSAP lesions. We report the case of a 76-year-old woman with DSAP who developed Bowen's disease, actinic keratosis, and keratoacanthoma on her face.
Aged ; Atrophy ; Bowen's Disease ; Carcinoma, Squamous Cell ; Female ; Humans ; Keratoacanthoma ; Keratosis, Actinic ; Porokeratosis* ; Skin*

No abstract available.
Porokeratosis*

No abstract available.
Humans ; Porokeratosis* ; Vitiligo*

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.
Clone Cells ; Female ; Humans ; Immunosuppression ; Keratinocytes ; Middle Aged ; Phototherapy* ; Porokeratosis* ; Psoriasis* ; Sunlight ; Transplants ; Ultraviolet Rays ; Ultraviolet Therapy

The rate of malignant transformation in porokeratosis (PK) lesions is approximately 7.5%, and linear PK demonstrates the highest rate of malignancy. An 83-year-old woman presented with a rapidly enlarging mass on her left arm. Variably sized erythematous scaly patches were scattered across the left half of her trunk and arm. Additionally, generalized variably sized brownish annular patches with a hyperkeratotic outer ring were observed on her face, trunk and bilateral arms. A skin biopsy was performed on 3 lesions-a yellowish to erythematous appearing mass, an erythematous scaly patch, and a brownish annular patch. Histopathological evaluation of these 3 lesions revealed squamous cell carcinoma, actinic keratosis, and PK, respectively. The final diagnosis was disseminated superficial PK with linear PK on the left side of the body, and actinic keratosis and squamous cell carcinoma confined to the linear PK lesions. We report a case which represents the progressive and stepwise malignant transformation of PK into squamous cell carcinoma.
Aged, 80 and over ; Arm ; Biopsy ; Carcinoma, Squamous Cell ; Diagnosis ; Female ; Humans ; Keratosis, Actinic ; Porokeratosis* ; Skin

No abstract available.
Humans ; Porokeratosis

OBJECTIVETo analyze the clinical and genetic features of 9 ethnic Han Chinese patients with disseminated superfacial actinic porokeratosis (DSAP).

METHODSGenomic DNA was extracted from peripheral blood samples collected from the patients. PCR and direct sequencing were carried out for five patients from a family, 4 sporadic cases, and 120 healthy controls to identify potential mutations of four genes (MVK, MVD, PMVK, FDPS) involved in the mevalonate pathway as well as SLC17A9, SSH1, and SART3 genes. Pathogenecity of suspected mutations were assessed with SIFT, and Polyphen-2 scores.

RESULTSA c.746T>C mutation was identified in the family and two sporadic cases, while a c.875A>G mutation was identified in another sporadic case. No mutation was identified in the remainder genes among all patients. Scoring has suggested that the c.746T>C and c.875A>G mutations of the MVD gene are probably pathogenic.

CONCLUSIONc.746 T>C and c.875A>G of the MVD gene are most common mutations. Skin rashes of the patients have a strong connection with the sunlight, albeit a significant difference among patients was discovered.

Aged, 80 and over ; Case-Control Studies ; Female ; Humans ; Male ; Middle Aged ; Mutation ; genetics ; Pedigree ; Porokeratosis ; genetics