Angiokeratoma of Tongue.

A 9 year old male presented with diffuse swelling on his tongue since childhood associated with intermittent bleeding. On examination there was multiple, sessile, firm, pinkish, erythematous, shiny papules on both dorsal and ventral surface of the tongue. Angiokeratomas are vascular malformations of capillaries characterized clinically by asymptomatic, solitary or multiple, keratotic papules or plaques, and histologically by benign vascular ectasia of the papillary dermis. Treatment usually consists of local excision and intralesional injection of steroids.

Cutaneous vascular lesions and their management in Indian setting.

Indian skin with its broad range of skin color and complexion differs in the presentation and management of cutaneous vascular lesions. Common congenital and acquired vascular lesions are discussed with respect to the epidemiology, clinical presentation, and management strategies in an Indian setting. An effort has been made to review Indian case reports and studies of cutaneous vascular lesions, potential possible modification in the conventional treatment considering resource constraints, cultural practices, availability and cost-effectiveness of the laser and light sources, camouflage techniques, and sclerotherapy. The review concludes with emphasis on the need of availability of cost-effective, quality equipment customized to Indian skin and quality studies and trials for the management of cutaneous vascular lesions in an Indian setting.

[A new location of angiokeratoma circumscriptum]. / Une nouvelle localisation de l'angiokératome circonscrit næviforme.

BACKGROUND: Angiokeratomas are papular telangiectasias having a common histology of ectasia of the superficial dermal vessels surmounted by a hyperkeratotic epidermis. PATIENTS AND METHODS: The patient was a 9-year-old girl born of non-consanguineous parents after a well-followed pregnancy with problem-free delivery at term. From birth, she had a tumefaction of the left side of the nose and the left half of the upper lip that gradually increased in size without obstructing the nasal orifice and bled easily. Examination revealed the presence of tumefaction of the left nostril and the left half of the upper lip projecting towards the contralateral side especially in the nose. It was soft and painless, with the presence at the surface of dull red keratotic papules of 1 to 2 mm in diameter. Examination of the nasal mucosa revealed the same appearance of papules. DISCUSSION: Angiokeratoma circumscriptum is a rare congenital malformation, the rarest of five types. Since its initial description in 1890, few cases have been reported. However, female predominance has been noted with a male/female sex ratio of 1/3. It appears to be due to a genetic mutation that is probably autosomal, but the site of which is still unknown. In view of the special features of this case, several diagnoses were suggested, including Rendu Osler's disease, superficial lymphangioma and verrucous angioma. CONCLUSION: The particularity of this case is that it includes the first description of this site, which posed a therapeutic problem, especially concerning the choice of laser type to be used.

Genital Rejuvenation of Scrotal and Vulvar Angiokeratomas: Laser Therapy of Scrotum and Vulva Lesions.

Genital rejuvenation requires medical and procedural treatments that can improve the morphology or aesthetics of the scrotum and vagina. Angiokeratomas are benign vascular lesions that my occur on the penis, scrotum, and vulva. These lesions can be of either cosmetic concern or medical significance if they bleed. Laser therapy can be successfully utilized for the treatment of genital angiokeratomas in men and women.

Report of two angiokeratoma of Fordyce cases treated with a 1064 nm long-pulsed Nd:YAG laser.

Various laser treatment modalities for angiokeratomas have been reported in the literature. A 60-year-old male patient presented for the treatment of multiple angiokeratomas of the scrotum and a 56-year-old female patient presented for the treatment of vulvar angiokeratomas. A 1064 nm long-pulsed Nd:YAG laser was used to treat the patients' lesions. The patients received two laser treatment sessions at an interval of 2 months. Nd:YAG laser treatment yielded successful results in the treatment of our patients' scrotal and vulvar angiokeratomas following two application sessions. We suggest that a long-pulsed Nd:YAG laser is a safe and effective method for the treatment of angiokeratoma of Fordyce.

Adolescent Vulvar Angiokeratoma Associated with Lichen Sclerosus.

BACKGROUND: We present an adolescent with multiple vulvar angiokeratomas within a background of lichen sclerosus. CASE: A 13-year-old girl presented with vulvar pruritus and wart-like vulvar lesions. Four lesions were resected because of discomfort and uncertainty of the diagnosis. Pathology revealed angiokeratomas with chronic inflammation suggestive of lichen sclerosus. Postoperatively, pruritus continued in the largest excised lesion, which was associated with lichen sclerosus, and symptoms were treated successfully with topical steroids. SUMMARY AND CONCLUSION: Vulvar angiokeratomas are asymptomatic red papular lesions and are rare in the female adolescent population. In this case, the pathology revealed the rare co-occurrence of angiokeratomas and lichen sclerosus. Biopsies of vulvar vascular lesions in symptomatic adolescents are recommended. Vulvar angiokeratomas might manifest rare genetic disease in otherwise asymptomatic female patients and warrant further follow-up.

Angiokeratomas of Fabry successfully treated with intense pulsed light.

Fabry disease (FD) is a rare X-linked lysosomal storage disorder resulting from the deficient activity of the enzyme alpha-galactosidase A. Angiokeratomas (AKs) are a frequent manifestation of this disease. They usually become apparent during childhood and can cause important cosmetic disability. Current treatment of this feature in the setting of FD has been mainly based on the application of laser systems, namely the argon laser, the variable pulse width 532-nm Nd:YAG laser, the 578-nm copper vapor laser and the flashlamp-pumped pulsed dye laser. We report the case of a 31-year-old Caucasian woman with a clinical and molecular (GLA p.R118C) diagnosis of FD, presenting multiple AKs scattered over the buttocks and thighs. She was treated with 10 sessions of intense pulsed light (IPL), with a 4-8-week interval between them. An almost complete clearance of the lesions was obtained, with no scars or significant complaints. No recurrence occurred during a 12-month follow-up period. The IPL source can be considered a suitable, effective and safe treatment modality for these cutaneous lesions that typically affect patients with FD, with no need for local anesthesia and with very satisfactory cosmetic results. To our best knowledge, there are no reports in the literature of Fabry's AKs treated with IPL.

Fabry disease: recognition and management of cutaneous manifestations.

Fabry disease (angiokeratoma corporis diffusum universale) is a rare, X chromosome-linked lysosomal storage disease. The deficient enzyme, alpha-galactosidase A (alpha-gal A), is responsible for the accumulation of neutral glycosphingolipids within vascular endothelial lysosomes of various organs, including skin, kidneys, heart, and brain. The disease manifests primarily in affected hemizygous men and to some extent in heterozygous women ('carriers'). The diagnosis of Fabry disease is made in hemizygous males after the detection of the presence of angiokeratomas, irregularities in sweating, edema, scant body hair, painful sensations, and of cardiovascular, gastrointestinal, renal, ophthalmologic, phlebologic, and respiratory involvement. A deficiency of alpha-gal A in serum, leukocytes, tears, tissue specimens, or cultured skin fibroblasts further supports the diagnosis in male patients. Since heterozygous women show angiokeratomas in only about 30% of cases and may have alpha-gal A levels within normal range, genetic analysis is recommended. Current treatment of angiokeratomas of Fabry disease is based mainly on the use of laser systems, including variable pulse width 532nm Neodymium:Yttrium-Aluminum-Garnet (Nd:YAG) laser, 578nm copper vapor laser, and flashlamp-pumped dye laser. When cutaneous and mucous glands are affected, restrictions may be required with regard to the time spent in a warm climate and the amount time spent working or on sporting activities, and may necessitate the use of topical and systemic antiperspirant agents, and topical application of artificial lacrimal fluid and saliva, respectively. For the future, new treatment modalities, including enzyme replacement therapy, substrate deprivation strategies, and gene therapy offer extraordinary options for the cutaneous and visceral lesions in patients with Fabry disease.

Cutaneous complications of Anderson-Fabry disease.

Anderson-Fabry disease is an X-linked lysosomal storage disorder caused by a defect in the α-galactosidase A gene, which leads to the deficiency of the hydrolytic enzyme α-galactosidase A. The consequent inability to catabolize glycosphingolipids causes progressive accumulation of globotriaosylceramide in the vascular endothelium throughout the body. Fatalities in the classical phenotype may usually occur as a consequence of cerebral, cardiac or renal disease. Dermatological manifestations are a relevant feature of Fabry disease and include angiokeratomas, telangiectasiae, lymphedema, anhidrosis or hypohidrosis and pseudo-acromegalic facial appearance. The actual causal treatment for Fabry disease is the enzyme replacement therapy. Dermatologists have a key role, since cutaneous manifestations may lead to the diagnosis. This may help an early therapeutic intervention, reducing both morbidity and mortality.

Angiokeratoma of the vulva.

Angiokeratomas are benign tumors characterized by ectasia of blood vessels in the papillary dermis associated with acanthosis and hyperkeratosis of the epidermis. Dermatological examination of angiokeratomas of Fordyce is characterized by papular keratotic lesions of erythematous-violet color. They are more common in the scrotum, and vulvar involvement is rarely reported. Histopathology is particularly important to distinguish them from other benign and malignant tumors. The article reports the case of a middle-aged black woman with a history of chronic constipation, varicose veins of the lower limbs and cesarean section performed 20 years ago who had had multiple vulvar angiokeratomas for three months.

Angiokeratoma of clitoris: a rare lesion in an adolescent girl.

Angiokeratoma is a benign vascular lesion. It is the result of dilation of ectatic subdermal vessels and congested capillaries. Weakness of the vessel walls, either from acquired or congenital reasons, can cause formation of the lesion. Angiokeratoma is more common in males, in whom it forms on the scrotal wall. An equivalent form can occur in females, generally on the vulva. The clitoris is an extremely rare location. We present the case of a 14-year-old girl with a clitoral angiokeratoma. To our knowledge, this is the first presented case of clitoral angiokeratoma in a child in English published reports.

Angioceratoma da vulva / Angiokeratoma of the vulva

Os angioceratomas são tumores benignos caracterizados por ectasia dos vasos sanguíneos da derme papilar associada à acantose e hiperceratose da epiderme. Ao exame dermatológico, os angioceratomas de Fordyce caracterizam-se por lesões papulo-ceratóticas de coloração eritemato-violácea. São mais comuns no escroto, sendo raramente relatada a localização vulvar. A histopatologia é particularmente importante para difer enciá-los de outros tumores benignos e malignos. O artigo relata o caso de uma mulher negra de meia idade com história de obstipação intestinal, varizes de membros inferiores e cesárea há 20 anos que apresentava múltiplos angioceratomas vulvares há três meses.

Angiokeratomas are benign tumors characterized by ectasia of blood vessels in the papillary dermis associated with acanthosis and hyperkeratosis of the epidermis. Dermatological examination of angiokeratomas of Fordyce is characterized by papular keratotic lesions of erythematous-violet color. They are more common in the scrotum, and vulvar involvement is rarely reported. Histopathology is particularly important to distinguish them from other benign and malignant tumors. The article reports the case of a middle-aged black woman with a history of chronic constipation, varicose veins of the lower limbs and cesarean section performed 20 years ago who had had multiple vulvar angiokeratomas for three months.

[Acral pseudolymphomatous angiokeratoma of children (APACHE)]. / Akrale pseudolymphomatöse Angiokeratome der Kindheit (APACHE).

Acral pseudolymphomatous angiokeratoma of children (APACHE) is a pseudolymphoma of posttraumatic origin which was first described in 1988. We report on a female patient with APACHE following a traumatic abrasion on her foot. Reddish papules developed that histologically were characterized by a wedge-shaped inflammatory infiltrate rich in capillaries. Conservative treatment was not successful.