Fabry disease (FD) is a multisystemic X-linked lysosomal storage disease that presents with angiokeratomas (AKs). Our objective was to investigate the clinical and morphologic features of AKs and to present two experimental techniques, multispectral imaging (MSI) and non-linear microscopy (NLM). A thorough dermatological examination was carried out in our 26 FD patients and dermoscopic images (n = 136) were evaluated for specific structures. MSI was used for the evaluation of AKs in seven patients. NLM was carried out to obtain histology samples of two AKs and two hemangiomas. Although AKs were the most common manifestation, the majority of patients presented an atypical distribution and appearance, which could cause a diagnostic challenge. Dermoscopy revealed lacunae (65%) and dotted vessels (56%) as the most common structures, with a whitish veil present in only 25%. Autofluorescence (405 nm) and diffuse reflectance (526 nm) images showed the underlying vasculature more prominently compared to dermoscopy. Using NLM, AKs and hemangiomas could be distinguished based on morphologic features. The clinical heterogeneity of FD can result in a diagnostic delay. Although AKs are often the first sign of FD, their presentation is diverse. A thorough dermatological examination and the evaluation of other cutaneous signs are essential for the early diagnosis of FD.

BACKGROUND: Angiokeratoma of Fordyce is typically asymptomatic, blue-to-red papules with a scaly surface located on the scrotum, shaft of penis or labia majora. They can be treated with some locally destructive treatment modalities such as excision, electrocoagulation, cryotherapy and laser.
OBJECTIVE: To compare the effects of the pulsed dye laser versus long pulsed Nd:YAG laser in the treatment of angiokeratoma of Fordyce.
METHODS: Twenty tow patients with angiokeratoma of Fordyce were included in this study. All participants received three sessions of pulsed dye laser on the selected side or part of lesional area and long pulsed Nd:YAG laser on the other side or part of lesional area. Two dermatologists independently evaluated the photographs of the baseline and two-month follow-up after last session using a grade system in which treatment response was categorized into six grades.
RESULTS: Both PDL and long pulsed Nd:YAG laser revealed statistically significant improvements in angiokeratoma of fordyce. Comparatively, there was a statistical difference between them (overall mean improvement with PDL, 61.8%, versus Nd:YAG, 77.63%; p < 0.005).
CONCLUSIONS: Both PDL and 1064-nm Nd:YAG laser are effective and safe in the treatment of angiokeratoma of Fordyce with better response in Nd:YAG laser than pulsed dye laser.

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BACKGROUND: Angiokeratomas are vascular anomalies in which the clinical, histological, and immunohistochemical characteristics are insufficient to determine whether the lesion is a vascular neoplasm or vascular malformation, and their exact origin is also uncertain. To further clarify the nosology of angiokeratomas, we studied 14 cases.
OBJECTIVE: To analyze immunohistochemical characteristics of angiokeratomas to gain further insight into its histogenesis.
METHODS: We carried out a retrospective review of the histopathology files. Immunohistochemical expression for Wilms tumor 1 (WT1), GLUT1, D2-40, podoplanin, Prox1, and ERG1 was performed in 14 cases.
RESULTS: None of the lesions showed cytoplasmic immunoreactivity for WT1. GLUT1 resulted to be negative in 13 cases. All 14 cases in our series showed some expression with at least 1 lymphatic marker. In 12 cases, the positivity was diffuse and strong for ERG1.
CONCLUSIONS: Angiokeratomas are complex lesions with difficult classification. Angiokeratomas are best considered vascular malformations in children, according to the WT1-negative stain. The lymphatic component of angiokeratoma is demonstrated by positivity and/or focal expression for lymphatic markers (podoplanin and Prox1); however, a blood capillary component within the malformation cannot be excluded.

Acral pseudolymphomatous angiokeratoma of children (APACHE) is characterized by multiple angiomatous papules on the hands and feet in children. Here, we report a case of APACHE in a female patient followed up from 13 to 19 years of age with a dark red lesion on the center of the dorsum of the right thigh. Histologically, vacuolar alteration and exocytosis of lymphocytes, and specific dense cellular infiltration beneath the epidermis to the reticular dermis were found. On immunolabeling study, the lesion vessels were found to be positive for both the lymphatic endothelium-specific marker podoplanin and blood vessel-specific marker CD34. These findings suggested that APACHE is a type of vascular malformation.

One of the more frequent pathologic conditions that podiatric physicians are confronted with is plantar verrucae. Plantar verrucae have been studied extensively in terms of morphological features and incidence in the population at large and in patients with human immunodeficiency virus infection. Solitary angiokeratomas can be morphologically similar to plantar verrucae, presenting as hyperkeratotic pedunculated lesions. We present a unique case study of a 40-year-old man with human immunodeficiency virus with a painful solitary angiokeratoma masquerading as plantar verrucae. The lesion demonstrated clinical signs consistent with those highlighted in the literature for verrucae, namely, showing as red and black lacunae, punctuated hyperkeratotic epidermis. We propose that solitary angiokeratomas should be an important part of a podiatric physician\'s differential diagnosis in the lower extremity owing to the similarity of morphological features with plantar verrucae.

Angiokeratoma is a rare, benign skin lesion and a recognised complication of radiation therapy. Here we describe a case of extensive angiokeratoma of the groin and external genitalia resulting from external beam radiation to that area in a patient with penile carcinoma. Furthermore, we outline the management of this problem by surgical reconstruction.

BACKGROUND: Pseudolymphomatous angiokeratoma (PA), originally termed \'acral pseudolymphomatous angiokeratoma of children\', is a disorder characterized clinically by development of red nodules on the extremities and histologically by a subepidermal dense lymphocyte infiltrate.
METHODS: We report three cases of PA, with characteristically dense, nodular infiltrate composed predominantly of small lymphocytes, and thick-walled vessels.
RESULTS: Immunohistochemical investigation revealed a dense accumulation of CD20+ cells with CD3+ cells in one case. Infiltrate in the other two cases was mainly composed of CD3+ cells and a mixture of CD4+ and CD8+ cells, with a few cells expressing CD20.
CONCLUSIONS: Our immunohistological results reveal a wide spectrum of cellular infiltrate compositions ranging from T-cell to B-cell predominance.

OBJECTIVE: 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.
METHODS: Multicenter retrospective study.
METHODS: University hospitals in Spain, Italy, Argentina, New York City, and Austria.
METHODS: 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.
METHODS: Dermoscopic examination.
METHODS: 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.
RESULTS: 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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