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Background: The term "diffuse neonatal hemangiomatosis" has been used historically to describe multifocal vascular lesions affecting the skin and viscera in infants., Objective: We hypothesized that many cases reported as diffuse neonatal hemangiomatosis did not have infantile hemangiomas (IH), but represented more recently described neonatal vascular diseases., Methods: A literature search was performed using PubMed database (1950-2009) with the terms "neonatal hemangiomatosis," "benign hemangiomatosis," and "diffuse hemangiomatosis." A total of 180 articles were identified. Exclusion criteria included disease onset later than 3 years of age and absence of multifocal skin involvement. In all, 73 cases were selected and categorized into 3 groups: IH/probable IH; multifocal lymphangioendotheliomatosis with thrombocytopenia (MLT)/probable MLT; and multifocal vascular lesions, not otherwise specified., Results: Of the 73 cases, 43 had IH/probable IH, 17 had MLT/probable MLT, and 13 had multifocal vascular lesions, not otherwise specified. The clinical outcomes of these groups differed in that two of 43 (5%) patients with IH died whereas 11 of 17 (65%) patients with MLT died (odds ratio 37.6, confidence interval 5.6-387.6, P value < .0001)., Limitations: This was a literature-based meta-analysis, which inherently has limitations of incomplete and inconsistently presented information., Conclusions: Many cases reported in the literature as diffuse neonatal hemangiomatosis represent newly described multifocal vascular anomalies such as MLT, which has a strikingly higher mortality than IH. We propose the term "multifocal infantile hemangioma-with or without extracutaneous disease" instead of "diffuse neonatal hemangiomatosis" for multiple cutaneous IH. Accurate diagnosis of multifocal neonatal vascular lesions is imperative to facilitate appropriate evaluation, treatment, and prognosis., (Copyright © 2012 American Academy of Dermatology, Inc. Published by Mosby, Inc. All rights reserved.)

Benign lymphangiomatous papules of the skin are considered reactive lymphatic proliferations either caused by disruption of the lymphatic flow or tissue damage produced by operation or radiation therapy. We report a 72-year-old woman with umbilical papules and vesicle-like lesions that led to the diagnosis of a large ovarian fibroma. Histologic study revealed dilated lymphatic spaces manifesting an anastomosing and branched pattern in the papillary and reticular dermis dissecting collagen bundles. The vessels were lined by plump endothelial cells with foci of intravascular papillary endothelial cell hyperplasia. After the ovarian fibroma was removed by laparotomy, umbilical lesions almost disappeared, leaving small flesh-colored papules. A periumbilical dermatosis may herald certain intra-abdominal diseases including those of neoplastic derivation. A heightened awareness of this association may lead to an early diagnosis with a potential for improved patient outcome. Benign lymphangiomatous papules have not been previously described in association with an untreated tumor, without previous operation or radiotherapy. This case advocates for disruption of the lymphatic drainage as the probable pathogenetic mechanism.

Multifocal lymphangioendotheliomatosis with thrombocytopenia is an extremely rare disease. This condition manifests as diffuse congenital vascular lesions in the skin and gastrointestinal tract leading to severe gastrointestinal bleeding and thrombocytopenia. Histopathologic and immunohistochemical studies of vascular lesions demonstrate a lymphatic endothelial cell origin. Treatment often is not satisfactory. We herein describe a 4-week-old infant with this uncommon clinicopathologic entity.

Benign lymphangiomatous papules of the skin are benign lymphatic proliferations that may arise in the skin after operation or radiation therapy. We report a case of benign lymphangiomatous papules of the skin that occurred in a patient 4 years after undergoing radiation therapy to the chest for adenocarcinoma of the breast.

Background: Klippel-Trénaunay syndrome (KTS) is a rare congenital anomaly classically defined as the triad of vascular stain, soft tissue and/or bony hypertrophy, and venous varicosities., Objective: To determine whether the morphologic characteristics of the associated vascular stains in KTS are predictive of the presence of lymphatic involvement and/or complications., Setting: Outpatient dermatology practice, tertiary care medical center., Methods: We retrospectively reviewed all cases of KTS identified between January 1989 and September 2001 at the University of California San Francisco (UCSF) Department of Dermatology. Forty patients were identified. We further classified them by type of cutaneous vascular stain, either "geographic" or "blotchy/segmental." Patients were further classified as having definite, probable, possible, or no evidence of lymphatic disease. We also reviewed the charts for other possibly associated manifestations and complications of KTS., Results: Of those with sharply demarcated geographic stains (n=22), 21 had definite or probable evidence of lymphatic disease. Of those with blotchy port-wine stains (n=17), 16 had possible or no evidence of lymphatic disease (P <.001). Determination of the type of stain had 95% sensitivity and 94% specificity in differentiating the definite or probable presence of definite or probable lymphatic disease from possible or no evidence of lymphatic disease. Complications occurred in 19 (86%) of 22 patients with a geographic stain vs 7 of 17 (41%) with a blotchy/segmental stain (P <.003)., Conclusion: This study demonstrates that the presence of a geographic vascular stain is a predictor of the risk of both associated lymphatic malformation and complications in patients with KTS. Since these stains are present at birth, this clinical observation can help in identifying individuals with KTS at greatest risk for complications and in need of closer observation.

Acquired progressive lymphangioma is a very rare disorder that presents in childhood or early adolescence as a lymphatic proliferation that gradually enlarges. It most commonly involves an extremity and is typically characterized as an indurated plaque that may be brownish-red, violaceous, or yellow. We report the case of a 15-year-old boy with a 10-year history of acquired progressive lymphangioma on the sole.

Kaposiform hemangioendothelioma is a locally aggressive, endothelial-derived spindle cell neoplasm that occurs exclusively in infants and adolescents. Lesions are characterized by rapid growth and extension, and are often associated with Kasabach-Merritt syndrome and lymphangiomatosis. Clinically nonspecific, they can appear as tender rapidly expanding red plaques, nodules, grouped papules, or telangiectasias. The histology is distinctive, however, as it combines features of tufted angioma, progressive lymphangioma, and Kaposi's sarcoma in a characteristic pattern. We describe a patient with kaposiform hemangioendothelioma currently controlled with systemic prednisone.

This second part of our review about vascular proliferations summarizes the clinicopathologic features of the cutaneous vascular hyperplasias and benign neoplasms. Hyperplasias comprise a heterogeneous group of vascular proliferations that eventually show a tendency to regression. Angiolymphoid hyperplasia with eosinophilia is included within the group of hyperplasias because of its historical denomination and its reactive nature, probably as a consequence of an arteriovenous shunt, although usually the lesions do not regress. Pyogenic granuloma, bacillary angiomatosis, intravascular papillary endothelial hyperplasia, and pseudo-Kaposi's sarcoma qualify as vascular hyperplasias because they regress when the stimulus that initiated them is removed. Benign neoplasms form a large group of hemangiomas with distinctive clinicopathologic characteristics, although some of them are of recent description and may produce diagnostic difficulties. We classified cutaneous benign vascular neoplasms according to their cell lineage of differentiation, for example, endothelial, glomus cell, and pericytic differentiation. Subsequent categories are established according to the size of the involved vessels (capillaries, venules and arterioles, or veins and arteries) or the nature of the proliferating vessels (blood or lymphatic vessels). Capillary and cavernous hemangiomas have been the terms classically used to name the most common variants of benign vascular neoplasms (i.e., infantile hemangiomas), but they are not the most appropriate denominations for these lesions. First, these names are not contrasting terms. Furthermore, most of the socalled "cavernous" hemangiomas are not hemangiomas (neoplasms) at all, but venous malformations. The most important conceptual issue is that, at any point in time, a particular hemangioma has its own histopathologic pattern throughout the depth of the lesion. For these reasons, we classified hemangiomas into superficial and deep categories. Some of the lesions reviewed have been recently described in the literature, and they may histopathologically mimic lesions of Kaposi's sarcoma; these include targetoid hemosiderotic hemangioma, microvenular hemangioma, tufted hemangioma, glomeruloid hemangioma, kaposiform hemangioendothelioma, spindle-cell hemangioendothelioma, and benign lymphangioendothelioma. In each of these lesions, we update and emphasize those clinical and histopathologic features that are helpful for differential diagnosis with lesions of authentic Kaposi's sarcoma in any of its three stages of development (patch, plaque, or nodule).

We describe a 40-year-old white man with a red-brown, indurated plaque on the proximal aspect of his right thigh. The lesion had been present since birth, and the patient had a 20-year clinical history of recurrent cellulitis in the same area. The histopathologic features of the lesion included permeation of the dermis by flattened, endothelium-lined channels without cellular atypia, hemorrhage, or inflammation. The endothelial cells were stained intensely with monoclonal antibody anti-CD34 (clone MY10). In addition, antibodies to factor VIII antigen, HLA-DR, smooth muscle actin, ICAM-1, and the lectin Ulex europaeus labeled the luminal cells. The basement membrane of the channels stained with anti-type IV collagen and laminin. Desmin-positive cells were abundant adjacent to the channels. Factor XIIIa stained both mononuclear cells and occasional dendritic cells in the perivascular area. Ki-67 immunolabeling could not be demonstrated on fresh or frozen tissue. Electron microscopy revealed the presence of both tight junctions and a well-formed, continuous basement membrane but the absence of Weibel-Palade bodies.

Acquired progressive lymphangioma is rare and can histologically mimic well-differentiated angioendothelioma. In a 9-year-old boy with acquired progressive lymphangioma, we demonstrated that the neoplasm consists of at least a vascular component and a smooth muscle component. The presence of type IV collagen around many vascular channels suggests that an intermediate stage of differentiation between blood vessels and lymphatics may be present. Our findings indicate that acquired progressive lymphangioma is most likely a complex hamartoma composed of vascular channels and smooth muscle.

We have studied eight cases of an acquired lymphatic endothelial lesion for which we propose the name "benign lymphangioendothelioma." The lesions developed as solitary, slowly extending, erythematous macules and plaques, usually occurring on the extremities or the shoulders in adolescents or adults. The characteristic histopathologic feature is permeation of the dermal collagen by flattened, endothelium-lined channels and spaces. Hemorrhage, iron deposition, and inflammation were not part of the lesion. Ulex europaeus agglutinin I labeled the lesional endothelial cells consistently, but factor VIII-related antigen labeling was negative. This histologic pattern and the special studies suggested a lymphatic lesion. Surgical excision, performed in six patients, was not followed by recurrence.

A 5-year-old Japanese boy, who developed dark brown, erythematous lesions (two separate lesions) on his face, scalp, and left upper arm within 2 years, was reported. Biopsy specimens revealed many dilated channels lined by a single layer of endothelial cells without obvious atypicality throughout the dermis. These channels split collagen bundles, giving a "dissection of collagen" appearance. From these clinical and histopathologic findings, we have diagnosed these lesions as acquired progressive lymphangioma. Our patient is the youngest reported patient in the literature, and we were interested in that the two lesions developed separately following previous trauma and they had the tendency to regress following oral prednisolone therapy.