Vulvar angiokeratoma

Nomelini, Rosekeila Simões; Pansani, P. L.; Guimarães, Patrícia Dias Neto; Martins-Filho, Agrimaldo; Barcelos, Ana Cristina Macêdo; Murta, Eddie Fernando Cândido

doi:10.3109/01443611003698703

Cited by 6 publications

(4 citation statements)

References 6 publications

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“…Vulvar angiokeratomas usually affect women in their reproductive years up to age 50 (Nomelini et al, 2010), but most commonly between ages 20 and 40 years (Kontogianni-Katsaros et al, 2006). Primarily located on the labia majora, and more rarely on the clitoris, vulvar angiokeratomas may appear as solitary or multiple, unilateral or bilateral lesions, however most are multiple, unilateral, and located on the left side of the vulva (Kontogianni-Katsaros et al, 2006).…”

Section: Discussionmentioning

confidence: 98%

Concurrent diagnosis of vulvar angiokeratoma with recurrent squamous cell carcinoma after radical vulvectomy and radiation therapy

Bhagan

Knaus

2012

Gynecologic Oncology Case Reports

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Section: Discussionmentioning

confidence: 98%

Concurrent diagnosis of vulvar angiokeratoma with recurrent squamous cell carcinoma after radical vulvectomy and radiation therapy

Bhagan

Knaus

2012

Gynecologic Oncology Case Reports

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“…Similar lesions are found in older women, where predisposing factors such as pregnancy, vulval varicosity and hysterectomy were found in 54% of the patients. Rare factors include vascular malformations or results of radiotherapy for genitourinary malignancy 51 …”

Section: Angiokeratoma In Other Rare Lysosomal Storage Disordersmentioning

confidence: 99%

“…Rare factors include vascular malformations or results of radiotherapy for genitourinary malignancy. 51 Angiokeratoma of Mibelli usually presents as grouped asymptomatic blue-black papules on the dorsum of hands and feet in females, manifesting typically between 10 and 15 years of age. There may be a familial predisposition and an association with chilblains, acrocyanosis or trauma.…”

Section: Angiokeratoma In Other Rare Lysosomal Storage Disordersmentioning

confidence: 99%

Angiokeratoma: decision-making aid for the diagnosis of Fabry disease

Zampetti

Orteu

Antuzzi

et al. 2012

British Journal of Dermatology

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Isolated angiokeratomas are common benign cutaneous lesions, generally deemed unworthy of further investigation. In contrast, diffuse angiokeratomas should alert the physician to a possible diagnosis of Fabry disease, a rare X-linked lysosomal storage disorder, characterized by α-galactosidase deficiency. Glycosphingolipids accumulate in cells throughout the body resulting in progressive multi-organ failure. Difficulties are encountered when trying to interpret the significance of angiokeratomas because they may also occur in other lysosomal storage disorders and rarely in an isolated manner in Fabry disease. We present an algorithm for the classification of angiokeratomas which might prove useful for the diagnosis and management of Fabry disease. Assessment of the clinical features and location of the lesions, personal and family history, skin biopsy, dermoscopy and electron microscopy imaging are sequential steps in the diagnostic process. Assessing the deficiency of α-galactosidase enzyme activity is essential to confirm the diagnosis in males, while mutation analysis is always needed in females. Potentially this algorithm can change the current approach to patients when Fabry disease is suspected, thus improving the diagnostic strategy and management of this disorder. It remains to be decided whether the use of an algorithm might reduce the number of genetic consultations. As evidence has shown the efficacy of enzyme replacement therapy in halting progression of the disease before the onset of irreversible organ damage, it is advisable to aim at an early diagnosis in order to achieve timely initiation of effective treatment with benefits for patients and appropriate use of medical resources.

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“…Vulvar angiokeratoma occurs rarely angiokeratomas of the vulva are benign vascular lesions usually occurring in women during their reproductive age. [ 1 ] With reported incidence of 0.16% only. [ 2 ] On review of literature, only three cases of radiation-induced vulvar angiokeratoma could be found.…”

Section: Introductionmentioning

confidence: 99%

Radiation-induced vulvar angiokeratoma along with other late radiation toxicities after carcinoma cervix: A rare case report

et al. 2016

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Angiokeratoma including vulvar angiokeratoma is a very rare complication of radiation. Exact incidence is still unknown, we report a case that developed radiation-induced angiokeratoma of skin in the vulvar region along with other late radiation sequelae in the form of bone fracture, new bone formation, bone marrow widening, muscle hypertrophy, and subcutaneous fibrosis, 18 years after radiotherapy to the pelvic region for the treatment of carcinoma cervix. All these late radiation sequel are rare to be seen in a single patient, and none of the case reports could be found in the world literature.

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Vulvar angiokeratoma

Cited by 6 publications

References 6 publications

Concurrent diagnosis of vulvar angiokeratoma with recurrent squamous cell carcinoma after radical vulvectomy and radiation therapy

Concurrent diagnosis of vulvar angiokeratoma with recurrent squamous cell carcinoma after radical vulvectomy and radiation therapy

Angiokeratoma: decision-making aid for the diagnosis of Fabry disease

Radiation-induced vulvar angiokeratoma along with other late radiation toxicities after carcinoma cervix: A rare case report

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