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      Penile Angiokeratomas (PEAKERs) Revisited: A Comprehensive Review

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          Abstract

          Angiokeratomas are benign vascular lesions. Genital angiokeratomas, also referred to as Fordyce angiokeratomas, usually occur on the scrotum in men and the vulva in women. Penile angiokeratoma (PEAKER) is a subtype of genital angiokeratoma in men; clitoral angiokeratoma (CLANKER) is its embryologic analog in women. The PubMed database was used to search the following words: angiokeratoma, clitoris, genital, peaker, penile, penis, rejuvenation, scrotal, scrotum and vulva. The relevant papers and references cited in those papers that were generated by the search were reviewed. The purpose of this article is to summarize the features of PEAKERs. PEAKERs have been described in 54 men. They usually appeared in younger men and had been present for a mean duration of 4 years prior to the individual seeking medical attention. Only 39% of the men had angiokeratoma-associated symptoms: usually bleeding and increasing size and less often abrupt onset, pain and pruritus. The glans penis (55.5%) and the penile shaft (35%) were the most common sites of PEAKERs; the angiokeratomas were also located on the foreskin (5.5%) or both the glans penis and penile shaft (4%). Thirty seven percent of patients with glans penis PEAKERs only had angiokeratomas on the corona. Scrotal angiokeratomas were also present in 20% of patients with PEAKERs. A solitary PEAKER was observed in 32% of the men. Most of the PEAKERs were 1–5 mm in size. The PEAKERs presented as purple, red and/or blue papules; 70% of the men’s PEAKERs were more than one color. Clinical features often established the diagnosis; in addition, some of the men’s angiokeratomas were biopsied or evaluated with dermoscopy. Laser therapy, in 56% of the men, was the most common treatment modality. Less common interventions included electrocautery, radiofrequency and excision. PEAKER recurrence or persistence was observed after excision (two men) or cryotherapy (one man), respectively. Several of the men (27%) decided to observe their PEAKERs without treatment.

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          Most cited references51

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          A Review of Fabry Disease.

          Fabry disease (FD) is an X-linked lysosomal storage disease. A lack of alpha-galactosidase activity results in the accumulation of globotriaosylceramide in cells of various systems, leading to multi-systemic effects. The cutaneous hallmark of FD is a specific distribution of angiokeratoma. Other common symptoms include cornea verticillata, acroparesthesia, and sweating abnormalities. FD-specific symptoms, history, as well as examination of angiokeratoma can assist in the differential diagnosis. Enzyme replacement therapy is the current mainstay of treatment.
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            Fabry disease: Review and experience during newborn screening.

            Fabry disease (FD) is an X-linked lysosomal storage disease and is the result of mutation in the α-Galactosidase A gene; such mutations cause a deficiency in α-Galactosidase A enzyme and an accumulation of glycosphingolipid in tissue. Affected males with classic FD have little or no enzyme activity and have an early onset of symptoms and signs, including acroparesthesias, hypohidrosis, angiokeratomas, gastrointestinal dysfunction and/or a characteristic corneal dystrophy during childhood/adolescence. Males with late-onset FD who have residual enzyme activity develop progressive multi-systemic involvement that leads to renal failure and hypertrophic cardiomyopathy, as well as cerebrovascular disease; these events mostly occur during the fourth to seventh decades of life. Heterozygous females can develop vital organ damage that in turn causes severe morbidity and mortality; these symptoms may be as severe as those in affected males. For the treatable disease, this review aims to raise awareness of early recognition and further management of FD based on newborn screening. As newborn screening for FD has been implemented worldwide, it allows the early detection of individuals with Fabry mutations. Based on screening studies, the prevalence of the later-onset type FD is much higher than that of classical type FD. Newborn screening studies have also revealed that patients with FD may develop insidious but ongoing irreversible organ damage. The timing of enzyme replacement therapy, which is able to stabilize the progression of disease, is important in order to prevent irreversible organ damage. Therapies that may become available in the future include pharmacological chaperones and substrate reduction therapy, both of which are still under investigation as ways of improving the health of individuals with FD.
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              Dermoscopy of solitary angiokeratomas: a morphological study.

              To describe the dermoscopic structures and patterns associated with solitary angiokeratomas and to determine the sensitivity, specificity, positive predictive value, negative predictive value, and reproducibility of these dermoscopic features. Multicenter retrospective study. University hospitals in Spain, Italy, Argentina, New York City, and Austria. There were 256 patients total, and 32 specimens each of solitary angiokeratomas, melanocytic nevi, Spitz-Reed nevi, malignant melanomas, pigmented basal cell carcinomas, dermatofibromas, seborrheic keratoses, and other vascular lesions (19 angiomas, 7 pyogenic granulomas, 3 spider nevi, 2 lymphangiomas, and 1 venous lake) were consecutively collected from the laboratories of 8 hospitals. Diagnoses of all patients' lesions were confirmed histopathologically. Dermoscopic examination. The frequency, sensitivity, specificity, positive predictive value, negative predictive value, intraobserver agreement, and interobserver agreement of the different dermoscopic features associated with solitary angiokeratomas were calculated, and the differences were evaluated using the chi(2) or Fisher exact test. Six dermoscopic structures were evident in at least 50% of the solitary angiokeratomas: dark lacunae (94%), whitish veil (91%), erythema (69%), peripheral erythema (53%), red lacunae (53%), and hemorrhagic crusts (53%). Dark lacunae exhibited a sensitivity of 93.8% and a specificity of 99.1% (P<.001 for both), not being found in malignant melanomas or pigmented basal cell carcinomas. The positive predictive value was 93.8%, and the negative predictive value was 99.1%. The intraobserver agreement was perfect (kappa, 1.00), and the interobserver agreement was excellent (kappa range, 0.83-1.00) (P<.001 for both). Pattern 1, consisting of dark lacunae and whitish veil, exhibited a sensitivity of 84.4% and a specificity of 99.1% and was not found in malignant melanomas or pigmented basal cell carcinomas. The positive predictive value was 93.1%, the negative predictive value was 97.8%, the intraobserver agreement was perfect (kappa, 1.00), and the interobserver agreement was excellent (kappa range, 0.83-1.00) (P<.001 for all). Conclusion Dermoscopy is helpful in improving the diagnostic accuracy of solitary angiokeratomas and allows the observer to differentiate them from other cutaneous tumors such as malignant melanomas and pigmented basal cell carcinomas.
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                Author and article information

                Contributors
                mitehead@gmail.com
                Journal
                Dermatol Ther (Heidelb)
                Dermatol Ther (Heidelb)
                Dermatology and Therapy
                Springer Healthcare (Cheshire )
                2193-8210
                2190-9172
                6 June 2020
                6 June 2020
                August 2020
                : 10
                : 4
                : 551-567
                Affiliations
                [1 ]San Diego Family Dermatology, National City, CA USA
                [2 ]GRID grid.265117.6, ISNI 0000 0004 0623 6962, Touro University California College of Osteopathic Medicine, ; Vallejo, CA USA
                Article
                399
                10.1007/s13555-020-00399-3
                7367967
                32506249
                371478ad-9c97-4c10-9ce5-25f634760940
                © The Author(s) 2020

                Open Access This article is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License, which permits any non-commercial use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by-nc/4.0/.

                History
                : 31 March 2020
                Categories
                Review
                Custom metadata
                © The Author(s) 2020

                Dermatology
                angiokeratoma,clitoris,genital,peaker,penile,penis,rejuvenation,scrotal,scrotum,vulva
                Dermatology
                angiokeratoma, clitoris, genital, peaker, penile, penis, rejuvenation, scrotal, scrotum, vulva

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