Concurrent diffuse dermal angiomatosis and granuloma inframammary adultorum of the breast.

Diffuse dermal angiomatosis (DDA) is a cutaneous reactive angiomatosis. Typically presenting as ulcerated, erythematous, violaceous, or purpuric plaques on the breast or lower extremities, DDA is believed to be a reaction to tissue ischemia. Granuloma inframammary adultorum (GIA) is a type of irritant dermatitis of multifactorial etiology, clinically presenting as papules and nodules. Herein, we report an interesting rash presenting as fungiform papulonodules overlying a large violaceous plaque on the left breast. Biopsy revealed an exuberant epidermal proliferation and a diffuse and deep dermal proliferation, consisting of small slit-like blood vessels in between collagen bundles. In light of these clinical and histopathologic findings in the setting of an indurated plaque on a pendulous breast of a woman with multiple risk factors for local tissue ischemia, a diagnosis of concurrent diffuse angiomatosis of the breast (DDAB) and GIA was rendered. This case highlights the critical importance of clinicopathologic correlation in the diagnosis of multiple diagnostic entities.

Diffuse dermal angiomatosis.

Diffuse dermal angiomatosis (DDA) is a benign and rare acquired, cutaneous, reactive, vascular disorder. We report a rare case of a 43-year-old man who presented with a large (15-cm diameter), indurated, hyperpigmented plaque covering the left buttock for 6 years. This report further discusses DDA with a review of the literature, including its classification, epidemiology, pathophysiology, etiology, histopathology, differential diagnosis, and current therapeutic approaches.

Solitary mediastinal angiomatosis: report of two cases and review of the literature.

Angiomatosis is a rare benign vascular lesion, usually seen in females in the first two decades of life. It commonly involves the lower extremities. Angiomatosis of mediastinum is very rare and we report two such cases with a review of the literature on solitary mediastinal angiomatosis. Both of our patients were female, aged 34 and 57 years. One patient presented with left-sided subcutaneous supraclavicular swelling. Magnetic resonance imaging showed a mass extending from the left upper mediastinum to the left supraclavicular area. The other patient presented with pleural effusion. Computed tomography of the chest disclosed an infiltrative mass in the mediastinum. Both patients were treated by excision of the lesions, histologically confirmed to be angiomatosis. Despite the rarity of mediastinal angiomatosis, this clinicopathological entity must be taken into consideration when the treatment of mediastinal tumours is planned.

An unusual case of persistent pneumothorax.

We herein report a case of a 45-year-old white male who referred to the emergency department for a right pneumothorax. A chest tube was emergently placed. Due to incomplete lung reexpansion, the patient underwent a right thoracoscopy disclosing the presence of several kinky vessels consistent of localized pleural angiomatosis, and a talc pleurodesis was performed. Computed tomographic scan and angiography confirmed an anomalous vascular connection between systemic and pulmonary circulation. Thus, a vascular percutaneous transcatheter embolization of the abnormal vessel was successfully executed and the patient was discharged without consequence.

Diagnosis and management of benign, atypical, and indeterminate breast lesions detected on core needle biopsy.

Imaging abnormalities detected by mammographic screening often lead to diagnostic evaluations, with suspicious abnormalities subjected to image-guided core needle biopsy (CNB) to exclude malignancy. Most CNBs reveal benign pathological alterations, termed benign breast disease (BBD). Adoption of CNB presents challenges with pathologic classification of breast abnormalities and management of patients with benign or atypical histological findings. Patient management and counseling after CNB diagnosis of BBD depends on postbiopsy determination of radiologic-pathologic concordancy. Communication between radiologists and pathologists is crucial in patient management. Management is dependent on the histological type of BBD. Patients with concordant pathologic imaging results can be reassured of benign biopsy findings and advised about the future risk of developing breast cancer. Surgical consultation is advised for patients with discordant findings, symptomatic patients, and high-risk lesions. This review highlights benign breast lesions that are encountered on CNB and summarizes management strategies. For this review, we conducted a search of PubMed, with no date limitations, and used the following search terms (or a combination of terms): atypical ductal hyperplasia, atypical hyperplasia, atypical lobular hyperplasia, benign breast disease, cellular fibroepithelial lesions, columnar cell lesions, complex sclerosing lesion, core needle biopsy, fibroadenomas, flat epithelial atypia, lobular carcinoma in situ, lobular neoplasia, mucocele-like lesions, phyllodes tumor, pseudoangiomatous stromal hyperplasia, radial scar, and vascular lesions. The selection of references included in this review was based on study relevance and quality. We used additional articles culled from the bibliographies of retrieved articles to examine the published evidence for risk factors of BBD.

Diffuse dermal angiomatosis of the breast: clinical and histopathological features.

INTRODUCTION: Diffuse dermal angiomatosis is an entity in the spectrum of reactive angiomatoses characterized by erythematous plaques that mainly affect the lower extremities of patients with a personal history of peripheral vascular disease. Involvement of the breast is a rare event that has only been described as single cases in women with large breasts. OBJECTIVE: Our main aim is to report three rare cases of diffuse dermal angiomatosis of the breast and to better define their clinical, histopathological, and immunohistochemical characteristics. Comorbidities and management will also be discussed. METHODS: A retrospective search of patients with the diagnosis of diffuse dermal angiomatosis of the breast was made. Databases of three hospitals, Hospital 12 de Octubre (Madrid, Spain), Hospital La Fe (Valencia, Spain), and Clinica Dermatologica, University of Genoa (Italy), were included in the analysis. RESULTS: Three middle-aged women who were heavy smokers were found. Physical examination revealed several livedoid plaques on both breasts. Painful ulceration over the violaceous lesions was observed in two cases. Histological features included a diffuse proliferation of spindle-shaped endothelial cells with focal small vessel formation occupying the full thickness of the dermis with scanty extravasated erythrocytes, showing positivity for CD31, CD34, and SMA-α and negativity for D2-40. Management was focused on a strict control of comorbidities, especially on the cessation of smoking habit that led to a conspicuous improvement in all patients. CONCLUSIONS: We describe all those clinicopathologic features that define diffuse dermal angiomatosis of the breast, which should be considered a distinctive variant into the group of cutaneous angiomatoses. In our experience, a strict control of smoking habit must be the first step in the management of this entity.

[Retinal angiomatous proliferations]. / Retinale angiomatöse Proliferationen.

Retinal angiomatous proliferations, also known as type 3 neovascularisation, are a common entity amongst patients with age-related macular degeneration. Their prevalence is being estimated at around 12-15% in this group of patients. Certain funduscopic signs like an extravofeal, intraretinal haemorrhage, cystoid macular oedema or a retinal anastomosis of the lesion are considered to be pathognomonic. Verification of the diagnosis should be based on ICG angiography, although OCT is gaining popularity. Interestingly, RAP lesions seem to have distinctive demographic characteristics and respond differently to established therapies, differentiating them from regular type 1 or type 2 neovascularisation. Current therapies of choice are VEGF inhibitors. Nonetheless, combination therapies, combining different approaches like anti-VEGF treatment and photodynamic therapy, have received more attention recently.

Primitive retinal vascular abnormalities: tumors and telangiectasias.

Primitive retinal vascular abnormalities are benign conditions of the retinal circulation that comprise vascular tumors and telangiectasias. The principal vascular tumors of the retina include retinal capillary hemangioma, cavernous hemangioma of the retina, racemose hemangiomatosis of the retina and retinal vasoproliferative tumor, while primary retinal telangiectasias include Coats' disease, Leber's miliary aneurysms and idiopathic juxtafoveal telangiectasias. In most cases, these alterations result in significant visual impairment due to exudation determined by the structural abnormalities of the retinal vasculature. The aim of this review is to assess the different clinical and diagnostic features of the single pathological entities and to discuss the available treatment modalities including the onset of intravitreal antivascular endothelial growth factor therapy.

Spinal arteriovenous malformation associated with spinal metameric syndrome: a treatable cause of long-term paraplegia?

Cutaneomeningospinal angiomatosis, or Cobb syndrome, is a rare metameric developmental disorder presenting as an extradural-intradural vascular malformation that involves bone, muscle, skin, spinal cord, and nerve roots. A 14-year-old girl with a red nevus involving the T6-9 dermatomes on the left side of her back presented with a 5-year history of bowel and bladder incontinence, paraplegia, and lower-extremity sensory loss. Magnetic resonance imaging demonstrated a hemangioma in the T-8 and T-9 vertebral bodies and a spinal cord AVM nidus extending from T-6 to T-9. The AVM was successfully embolized and the patient regained lower-extremity strength, ambulation, and normal sphincter functions after 5 years of having been wheelchair bound. The authors report the restoration of ambulation after endovascular embolization of a large spinal AVM in a patient with long-standing paraplegia due to Cobb syndrome.

Revascularización percutánea de angiosomas específicos en isquemia crítica de la extremidad / Percutaneous revascularisation of specifi c angiosome in critical limb ischaemia

Introducción y objetivos. Analizar si la adquisición de fl ujo directo al angiosoma isquémico delpie infl uye en la cicatrización y en el salvamento de la extremidad ante isquemia crítica tratadamediante terapia endovascular.Material y métodos. Análisis retrospectivo de 34 extremidades (32 pacientes) consecutivas conisquemia crónica categoría 5 (n = 21; 61,8 %) y 6 (n = 13; 38,2 %) de Rutherford y tratadas exitosamentemediante técnica endovascular, entre 2007 y 2009. Elaboramos dos grupos: el grupo derevascularización directa o RD (la arteria revascularizada nutre directamente el angiosoma isquémico)y el grupo de revascularización indirecta o RI (la arteria revascularizada no nutre directamenteel angiosoma isquémico). Se analizó la cicatrización completa, el tiempo de cicatrizacióny la tasa de salvamento de la extremidad. El análisis estadístico incluyó la prueba deChi-cuadrado y tablas de supervivencia.Resultados. Se incluyeron 34 extremidades sometidas a recanalización (n = 27; 79,4 %), angioplastiasimple (n = 3; 8,8 %) y angioplastia con stent (n = 4; 11,8 %). El grupo RD incluyó 18(52,9 %) extremidades y el grupo RI 16 (47,1 %). Al comparar comorbilidades, no se encontrarondiferencias significativas. El 76,5 % (n = 26) de las heridas cicatrizaron completamente y no resultódiferente el tiempo total de curación (RD 157,8 días frente a RI 124,5 días). Al comparar eléxito de cicatrización completa (RD n = 14 [77,8 %] frente a RI n = 12 [73,7 %]) y la tasa de salvamentode extremidad al año (RD 89 % frente a RI 84 %) tampoco hubo diferencias.Conclusión. En nuestra serie, la revascularización directa o indirecta del angiosoma no infl uyeen la tasa de cicatrización y de salvamento de la extremidad(AU)

Introduction and objectives. To determine whether the acquisition of direct fl ow to ischaemicfoot angiosome affects healing and limb salvage in critical limb ischaemia treated byendovascular therapy.Material and methods. Retrospective analysis of 34 consecutive limbs (32 patients) with chronicischaemia, Rutherford category 5 (n = 21, 61.8 %) and 6 (n = 13, 38.2 %) and successfully treatedwith endovascular technique, between 2007 and 2009. We created two groups: the directrevascularisation group or DR (revascularised artery directly feeds the ischaemic angiosome)and the indirect revascularisation group or IR (revascularised artery does not directly feed theischaemic angiosome). The variables analysed were: complete healing, healing time and limbsalvage rates. Statistical analysis included Chi-square test and survival tables.Results. We included 34 limbs who underwent recanalisation (n = 27, 79.4 %), simple angioplasty(n = 3, 8.8 %) and angioplasty with stents (n = 4; 11.8 %). The DR group included 18 (52.9 %) limbsand the IR group 16 (47.1 %). We did not find significant differences when comparingcomorbidities. There was complete healing in 76.5 % (n = 26) of the wounds and there were nodifference in the total healing time (157.8 days in DR against 124.5 days in IR). There wereno differences when comparing the success of complete healing (DR n = 14 [77.8 %] against IRn = 12 [73.7 %]) and the limb salvage rate per year (89 % DR against 84 % IR).Conclusion. In our series, the direct or indirect revascularisation angiosome does not affect therate of healing and limb salvage(AU)

Pseudoangiomatous stromal hyperplasia (PASH): a brief review.

Pseudoangiomatous stromal hyperplasia (PASH) is a benign entity of the breast and typically found incidentally. It warrants thorough investigation in order to exclude more sinister pathology masquerading as this form of benign breast disease and can often be managed expectantly without the need for surgical intervention. We provide a brief review of the literature on PASH, discussing its clinicopathological features and management.

[Treatment of cutaneous vertebral medullary angiomatosis].

OBJECTIVE: Cutaneous vertebral medullary angiomatosis, also known as Cobb's syndrome, is a part of spinal arteriovenous metameric syndromes (SAMs), is derived from the vascular malformation triad of skin, bone, and spinal cord involvement. It is poorly managed with current treatment modalities. We reviewed the treatment of Cobb's syndrome series and summarized the experiences. METHODS: A total of 61 cases of Cobb's syndrome with spinal cord dysfunction were treated at our department from February 2003 to December 2007. The treatment followed the same strategy: First step-investigating the pathogenic mechanisms by symptom onset, MRI, angiography and the response to initial treatment. Second step-treating the problematic parts of the lesions with embolization alone, surgery alone or combination of both. Forty-eight cases were embolized, 3 cases treated with surgery and 10 cases treated with a combination of both. Nidus involving pathogenic mechanism for spinal cord were eliminated, completely and nearly disappeared in 36 cases and partially in 25 cases. All patients were followed up regularly. RESULTS: Among these 61 cases, the pathogenic mechanisms were identified as hemorrhage, mass effect, ischemia and venous hypertensive myelopathy. Two or more mechanisms could coexist in the same patient. More than 1-year's spinal cord function follow-up showed: excellent in 9 cases, good in 26, fair in 23 and worse in 3. CONCLUSION: Not all lesions of Cobb's syndrome can be or should be cured anatomically. Pathogenic mechanism should be analyzed carefully and the treatment should focus on the special cord-affecting targets. Long-term improvement or stabilization may be achieved.

Comparison of treatment of cherry angiomata with pulsed-dye laser, potassium titanyl phosphate laser, and electrodesiccation: a randomized controlled trial.

OBJECTIVE: To assess the comparative efficacy of energy treatments in resolving cherry angiomata. DESIGN: Rater-blinded randomized controlled trial. SETTING: Outpatient dermatology clinic in an urban referral academic medical center. PARTICIPANTS: Fifteen healthy adults aged 21 to 65 years were enrolled. Two eligible individuals who were approached declined to participate, and no one enrolled was withdrawn for adverse effects. INTERVENTIONS: For each participant, 3 areas on the torso were demarcated such that each area contained 4 cherry angiomata. Each area was then randomly assigned to receive 1 of the 3 treatments: pulsed-dye laser (PDL) (595 nm), potassium titanyl phosphate (KTP) laser (532 nm), or electrodesiccation. Two treatments spaced 2 weeks apart were delivered to each area. MAIN OUTCOME MEASURES: Standardized photographs from before treatment and 3 months after the last treatment were evaluated for color and texture on visual analog scales. RESULTS: Mean change in color was a significant improvement of 7.77 (P<.001), but there was no significant difference across treatment arms (P=.19). Mean change in texture was a significant improvement of 6.23 (P<.001), and the degree of textural change also differed across treatments (P<.001). In pairwise comparisons, cherry angiomata treated with electrodesiccation were significantly less improved than were those receiving KTP laser (P=.003) and those treated with PDL (P=.001). The effects of KTP laser and PDL on texture were not different (P=.50). CONCLUSIONS: Cherry angiomata can be effectively treated with electrodesiccation and with laser. Laser, especially PDL, may minimize the likelihood of treatment-associated textural change. Trial Registration clinicaltrials.gov Identifier: NCT00509977.

Current treatment options for retinal angiomatous proliferans (RAP).

Retinal angiomatous proliferation (RAP) accounts for 12-15% of all patients with neovascular age-related macular degeneration (NV-AMD). However, this subtype is often excluded from clinical trials aimed at assessing the efficacy of various treatment options for NV-AMD. Thus, there are no established protocols for the management of RAP. This review of current literature on RAP compares the outcomes of various treatment options for this condition and highlights the lack of clinical trials and paucity of long-term data on this relatively common condition.

[Laser photocoagulation and photodynamic therapy (PDT) with verteporfin for retinal angiomatous proliferation (RAP) in age-related macular degeneration (AMD)]. / Laserfotokoagulation und photodynamische Therapie (PDT) zur Behandlung der retinalen angiomatösen Proliferation (RAP) bei feuchter altersabhängiger Makuladegeneration (AMD).

BACKGROUND: Retinal angiomatous proliferation (RAP) is a subtype of neovascular age-related macular degeneration (AMD) with particularly bad prognosis. Diverse treatment modalities are performed. PATIENTS AND METHODS: This is a retrospective review on the treatment results of 41 consecutive patients from 1/2003 to 12/2005 with RAP stage 1-3 (Yannuzzi classification), who were treated with laser photocoagulation, photodynamic therapy (PDT) and intravitreal injection of triamcinolone acetonide (IVT). Follow-up was 12 months minimally. RESULTS: In RAP stage I complete closure of the vascular lesion in 14 / 22 eyes was achieved by 1.2 +/- 0.5 sessions of laser photocoagulation (4 x combined with IVT) and in 3/14 eyes with photodynamic therapy (2 +/- 0.5 sessions). In RAP stage II closure of the lesion was achieved by 3.2 +/- 0.6 sessions of photodynamic therapy in 6/14 eyes (4 x combined with IVT). In RAP stage III closure of the lesion was achieved by 3.2 +/- 0.4 sessions of photodynamic therapy in 0 / 5 eyes (3 x combined with IVT). A rip of the retinal pigment epithelium was observed in 2/14 eyes of RAP stage II and 2/5 eyes of RAP stage III. Visual acuity improved in 9/17 eyes with occlusion of RAP stage I. Without closure of the vascular lesion all eyes got legally blind (visual acuity 1/50 or less). CONCLUSIONS: Early detection and subsequent direct treatment of RAP stage I in AMD is recommended. In advanced stages anatomical closure of the vascular complex is rarely achieved and the risk is improved for development of tears in the retinal pigment epithelium and getting legally blind.

Micropulsed laser photocoagulation and intravitreal triamcinolone acetonide injection for the treatment of retinal angiomatous proliferation.

PURPOSE: To investigate visual acuity and fluorescein angiographic outcomes, as well as adverse events, associated with treatment of retinal angiomatous proliferation (RAP) with micropulsed laser photocoagulation and intravitreal triamcinolone acetonide injection. METHODS: In this retrospective, noncomparative, interventional, consecutive case series, the medical records of all patients treated for RAP with micropulsed laser photocoagulation (yellow or green dye; duration, 0.02-0.05 second; power adjusted to achieve a white burn of moderate intensity at the level of the RAP lesion in the retina) and intravitreal triamcinolone acetonide (4 mg/0.1 mL) injection between January 2003 and November 2004 were reviewed by one of four retina specialists at a single retina practice. Main outcome measures were visual acuity, leakage shown by fluorescein angiography, and adverse events. RESULTS: The study included 14 eyes of 13 patients (8 women and 5 men; median age, 83 years [range, 70-90 years]). Triamcinolone acetonide injection preceded laser treatment by a median duration of 7 days (range, 5-16 days) in 8 eyes, was performed on the same day as laser treatment in 2 eyes, and followed laser treatment by a median duration of 7 days (range, 7-28 days) in 4 eyes. Eyes were followed a median of 18 months (range, 12-27 months) after treatment with both laser and intravitreal triamcinolone injection. Compared with pretreatment visual acuity, vision at 12 months and the last follow-up examination was stable in 5 eyes (36%), improved by >or=2 lines in 6 eyes (43%), and worsened by >or=2 lines in 3 eyes (21%). The median visual acuity before treatment was 20/200 compared with 20/80 at 3 months after treatment (P = 0.02), 20/100 at 6 months after treatment (P = 0.16), 20/200 at 12 months after treatment (P = 0.73), and 20/100 (P = 0.63) at the last follow-up examination. For 13 eyes (93%), fluorescein angiography performed 6 months after administration of both laser and intravitreal triamcinolone injection demonstrated resolution of all leakage; one eye was treated with photodynamic therapy with verteporfin 6 months after treatment for persistent low-grade leakage. Elevated intraocular pressure (>25 mm Hg) occurred in 2 eyes at 2.5 months and 2 months after intravitreal triamcinolone acetonide injection; both eyes were treated with transient use of antiglaucoma eyedrops. Seven (78%) of 9 phakic eyes had cataract progression, and 5 of these 7 eyes underwent cataract extraction during the follow-up period. No other adverse events occurred. CONCLUSIONS: Micropulsed laser photocoagulation and intravitreal triamcinolone acetonide injection may be effective and safe treatment for RAP lesions.

Kasabach-Merritt syndrome caused by giant hemangiomas of the spleen in patients with Proteus syndrome.

Proteus syndrome is a rare hamartomous disorder with highly variable manifestations of disproportionate overgrowth of body parts, epidermal nevi and vascular malformations. We describe two patients who had typical clinical and imaging Proteus syndrome features. One of most characteristic findings in these two patients was that they had giant hemangiomas of the spleen that caused Kasabach-Merritt syndrome, demonstrating clinical bleeding and disseminated intravascular coagulation laboratory alterations. The present two cases are the first report of this complication in Proteus syndrome. The patients' bleeding tendency and hemostatic defects were completely corrected after they successfully underwent splenectomy.