Pseudoangiomatous stromal hyperplasia (PASH) presenting as axillary lump: case report and review of the literature.

Pseudoangiomatous stromal hyperplasia (PASH) is an uncommon benign mesenchymal breast lesion. There are extremely rare reports of PASH arising in accessory breast tissue. To date, in literature, fewer than 10 cases of PASH occurring in axillary region have been described. We report a case presenting as axillary lump in a young woman. A 20-year-old female presented to our surgical unit for a progressively growing and painful palpable mass of the right axilla for about a year. Before surgery an ultrasound was performed. The patient underwent local excision of the lesion under local anaesthesia. Through histological and immunohistochemical examination a pseudoangiomatous stromal hyperplasia (PASH) was diagnosed. At 6 months of followup the patient is free of disease. It is important to include PASH also in the differential diagnosis of axillary lumps. Histological examination of the surgical specimen and surgery represent, respectively, the mainstay for diagnosis and therapy.

Endothelial chimerism in chronic sclerotic-type chronic graft-versus-host disease (GVHD) and GVHD-associated angiomatosis.

Graft-versus-host disease-associated angiomatosis (GVHD-AA) is an uncommon manifestation of chronic GVHD consisting of friable vascular proliferations. Using fluorescence in situ hybridization, we demonstrate the presence of donor-derived endothelial cells within areas of GVHD-AA. This is the first documented occurrence of a benign neoplastic growth in relationship to a form of chronic GVHD.

Diffuse Dermal Angiomatosis of the Breast With an Apparent Etiology of Underlying Calcified Thrombosed Artery With Adjacent Fat Necrosis.

In this report, the authors present a case of diffuse dermal angiomatosis (DDA) with an underlying mass lesion of the breast, which proved to be a large calcified, thrombosed artery with adjacent fat necrosis. Histologically, DDA consists of hyperplastic vessels, which diffusely infiltrate the papillary and reticular dermis forming small vascular lumina. The condition is associated with various underlying conditions, many of which result in local tissue ischemia. In the past, DDA was most commonly reported on the lower extremities; however, it seems that this entity is more common on the breast than previously recognized. Various treatments have proven beneficial, including revascularization, oral corticosteroids, smoking cessation, and isotretinoin. In this case, our patient benefited from primary excision of the affected area.

Unusual cause of lower extremity wounds: Cobb syndrome.

Cobb syndrome (Cutaneomeningospinal Angiomatosis) is a rare segmental neurocutaneous syndrome associated with metameric cutaneous and spinal cord arteriovenous malformations (AVMs). In this syndrome, capillary malformation or angiokeratoma-like lesions are formed in a dermatomal distribution, with an AVM in the corresponding segment of the spinal cord. The spinal cord lesions can cause neurological disorder and paraplegia, which typically develop during young adulthood. We report a 32-year-old male with the Cobb syndrome associated with lower extremity painful wounds and acute-onset paraplegia due to metameric vascular malformations.

Diffuse dermal angiomatosis associated with severe atherosclerosis: two cases and review of the literature.

Diffuse dermal angiomatosis (DDA) is a rare condition characterized by endothelial proliferation in the reticular dermis. Several diseases have been associated with DDA, including peripheral arterial disease (PAD). We report two cases of DDA associated with PAD. Patient 1 was a 71-year-old woman, who presented with painful necrotic ulcerations on her trunk and a medical history of PAD. Skin biopsy revealed a dermal proliferation of endothelial cells, and despite medical treatment, she died 1 month later. Patient 2 was an 81-year-old man, who presented with an erythematous, bluish plaque of the shoulder. He was a heavy smoker, with severe PAD. Biopsy showed dermal capillary hyperplasia, with a few fibrin thrombi, and follow-up only was recommended. In both cases, laboratory tests and Doppler ultrasonography ruled out other thrombotic conditions and vascularitis. DDA is a rare complication of PAD, and the optimum medical treatment remains to be clarified, especially when revascularization has failed or is not possible, as in our cases.

Diffuse dermal angiomatosis associated with calciphylaxis in a patient with end-stage renal disease.

Diffuse dermal angiomatosis (DDA) represents a benign, acquired, reactive proliferation of vessels. DDA is clinically characterized by painful livedoid plaques with central ulceration, and the histopathologic hallmark is diffuse endothelial cell hyperplasia in the dermis. DDA has been rarely reported in association with calciphylaxis, a condition characterized by calcification of arterial walls with accompanying thrombosis and cutaneous necrosis. We present a case of a 72-year-old man with end-stage renal disease on peritoneal dialysis who presented with painful lesions on his legs, and was found to have DDA in the setting of calciphylaxis. The possible pathogenesis linking DDA and calciphylaxis is discussed.

Cutaneous reactive angiomatosis with combined histological pattern mimicking a cellulitis.

Cutaneous reactive angiomatoses (CRA) encompass a distinct group of rare benign reactive vascular proliferations that include reactive angioendotheliomatosis, diffuse dermal angiomatosis and reactive intralymphatic histiocytosis. The etiology of these conditions, often associated with either localized or systemic diseases, is poorly understood. We report a 72-year-old woman who presented giant diffuse cellulitis-like plaques on the right lower limb and the pelvis and a reduction of her general condition with fever. Light microscopy studies revealed combined features of reactive angioendotheliomatosis, diffuse dermal angiomatosis and reactive intralymphatic histiocytosis. A small arteriovenous fistula of the right lower leg was thought to act as trigger. Systemic corticosteroids resulted in the clinical remission of the skin lesions. Our observation provides strong evidence that reactive angioendotheliomatosis, diffuse dermal angiomatosis and reactive intralymphatic histiocytosis, previously regarded as distinct forms of CRA, may show overlapping histopathological features and most likely represent facets of the same disease.

Progressive late-onset of cutaneous angiomatosis as possible sign of cerebral cavernous malformations.

BACKGROUND: Cerebral cavernous malformations (CCM) comprise enlarged capillary cavities in the central nervous system, with possible retinal or cutaneous vascular malformations. This condition is associated with CCM1, CCM2, and CCM3 gene mutations. OBJECTIVE: Cutaneous clinical, histological and cerebral MRI findings, including CCM1, CCM2, and CCM3 gene sequencing, of two unrelated, neurological symptom-free patients who consulted for late-onset of deep multiple cutaneous angiomatoid lesions, are described. RESULTS: The diagnosis of multiple cutaneous angiomatosis was confirmed and related to CCM as detected by MRI in both cases. Analysis of our patients showed normal nucleotide sequences of the genes proposed. CONCLUSIONS: A progressive late-onset of multiple, deep cutaneous venous malformations may indicate the need to investigate a potential coexistence of CCM by MRI. Early diagnosis and prompt treatment is required in these patients. The absence of CCM1, CCM2, and CCM3 mutations might indicate that different genes could be involved in the pathogenesis of these late-onset patients. Careful questioning about family history of CCM is important; our first patient's daughter had a history of cerebral cavernoma.

[Combined methods of treatment the congenital arterio-venous fistulas of peripheral vessels].

A 10-year experience and results of combined methods of surgical treatment of arterio-venous fistulas of peripheral vessels in 50 patients were analyzed. The patients were systematized on the basis of existing classifications, clinical manifestations of the disease, methods of invasive (ultrasound dopplerography and duplex scanning) and invasive (angiography) examinations. According to many authors no one of conventional methods of surgical treatment of arterio-venous fistulas which are used singly can be effective and must not be recommended as the most optimal. Stepwise employing of traditional operations and endovascular techniques are the main conditions for preventive measures of ischemic disorders in the limbs.

Diffuse dermal angiomatosis.

Diffuse dermal angiomatosis (DDA) is characterized clinically by painful erythematous lesions with ulcers and histologically by a benign, diffuse, and self-limited proliferation of tiny blood vessels in the superficial layers of the reticular dermis. Here we describe a case of DDA with leg ulcer. Erythematous lesions presented around the ulcer and angiogram revealed an occlusion of the superficial femoral artery. The erythematous lesions disappeared after revascularization. Although DDA is extremely rare, early correction of the ischemia in the peripheral artery should be taken into consideration.

Pseudoangiomatous Stromal Hyperplasia (PASH) of the Breast: An Uncommon Finding in an Uncommon Patient.

BACKGROUND Pseudoangiomatous stromal hyperplasia (PASH) is an uncommon benign breast lesion. CASE REPORT PASH is reported in a young female in treatment for neurological diseases with multi-drug therapy (clonazepam, valproate and risperidone). Her menstrual cycles are irregular, and she reached menarche very late. CONCLUSIONS The higher PASH prevalence in premenopausal woman (the majority of whom are actively taking oral contraceptive pills), in 24% to 47% of men with gynecomastia and during pregnancy supports a hormonal etiology; the interaction between clonazepam, valproate, risperidone and progesterone could increase the level of progesterone that could stimulate PASH growth.

Intravascular eosinophilic deposits-when common knowledge is insufficient to render a diagnosis.

In the course of daily sign-out, the diagnoses within a histopathologist's armamentarium are limited by the scope of the histopathologist's knowledge, that is, one cannot diagnose what one does not know. The subject of homogeneous intravascular eosinophilic deposits is used to illustrate this point. A histopathologist unaware that a tick bite reaction can induce intravascular eosinophilic deposits may misdiagnose the specimen as representing a manifestation of cryoglobulinemia. Furthermore, conventional teaching imparts that monoclonal cryoglobulinemia shows intravascular eosinophilic deposits (cryoprecipitates) histopathologically, whereas mixed cryoglobulinemia is histopathologically manifested as leukocytoclastic vasculitis. Although it is not well known, this is not always the case because mixed cryoglobulinemia may histopathologically present itself as intravascular eosinophilic deposits without leukocytoclastic vasculitis. In addition, it is not common knowledge that intravascular cryoprecipitates, when present, may be associated with an increased number of blood vessels. Examples of these phenomena are presented in conjunction with a discussion of relevant issues/lessons learned from such cases.

Angiomatosis and ectopic ossification in the dog myocardium after intramyocardial implantation of autologous bone marrow mononuclears in experimental coronary disease.

Disseminated ossification of the myocardium and severe angiomatosis were detected in sites of implantation of unseparated bone marrow mononuclear fraction cells in dogs with experimental chronic coronary disease. Minor immunocytochemical differences in cells of the mononuclear fraction adhering and not adhering to plastic were found. Significant differences in the expression of mRNA of chondro-osteogenesis genes (aggrecan, lumican, and osteopontin) in adherent and nonadherent cells were detected. The expression of aggrecan gene mRNA was 3-fold lower, of lumican gene 6-fold, and of osteopontin gene 11-fold lower in nonadherent cells compared to adherent fraction.

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.

Parkes Weber syndrome presenting as Stewart-Bluefarb acroangiodermatitis.

Parkes Weber syndrome (PWS) is a rare disorder characterised by arteriovenous (AV) fistula, along with capillary, lymphatic, venous malformations and limb hypertrophy. Stewart-Bluefarb syndrome is a variant of acroangiodermatitis, which is associated with congenital AV malformation/fistulas. It usually begins early in life, unilaterally over lower extremities presenting as violaceous to dusky coloured macules, papules or plaques with tendency to ulcerate. We are reporting a case of AV malformation fulfilling the triad of PWS and presenting with acroangiodermatitis.

Types of choroidal neovascularisation in newly diagnosed exudative age-related macular degeneration.

AIM: To describe the types and location of choroidal neovascularisation (CNV) in exudative age-related macular degeneration (AMD), including vascularised pigment epithelial detatchments (PED), and most recently described subtypes, such as retinal choroidal anasmostosis, also termed "retinal angiomatous proliferation" (RAP). METHODS: Prospective multicentre consecutive descriptive case series. A total of 207 consecutive cases of newly diagnosed exudative AMD undergoing fluorescein angiography (FA) were recruited by 7 French referral hospital-based or private centres. Indocyanine green angiography (ICG) also was performed, when judged necessary by investigators. Types and location of CNV were classified by two independent experts and adjudicated by a third when discordant. RESULTS: All patients had FA, while ICG was performed in 50% of subjects. A total of 17.6% had classic CNV only, 5.4% and 8.3% had predominantly and minimally classic CNV, respectively. Occult CNV could be classified in occult CNV without PED (32.7%) and occult CNV with PED, ie, vascularised PED (23.9%). RAP was observed in 15.1% of cases, and accounted for 30% of vascularised PED. In 5.8% of the cases there was haemorrhagic AMD and 4.8% had fibrovascular scars. Lesions were mainly subfoveal (80%). Agreement between the centre's ophthalmologist and the final validated expert classification was moderate (kappa = 0.52 for location and 0.59 for type of lesion). CONCLUSION: This study confirms that newly diagnosed cases of exudative AMD are mainly occult and subfoveal. RAP appeared as a common lesion in patients with newly diagnosed exudative AMD.