Lesions typically present during infancy or early childhood and are most commonly reported to persist and/or expand over time. Congenital presentations are rare, as are reports of spontaneous regression. [ncbi.nlm.nih.gov]

• Localized Pain

  We describe a case of a 11-year-old girl with a tufted angioma located in the trunk treated with pulsed-dye laser, showing important relief of local pain, and… CONTINUE READING References Publications referenced by this paper. [semanticscholar.org]

  The most common symptom is local pain, usually non responding for non-steroid anti-inflammatory drugs. Radiation therapy usually does not result calcification or the tumor regression, but significantly reduces the pain intensity ... [centerwatch.com]

  During laser application, the patient complained of pain, despite use of topic local anesthetic (lidocaine and prilocaine). [scielo.br]

  pain and swelling during the followup. [analesdepediatria.org]

• Lymphedema

  At the age of 14, the limb showed severe lymphedema and discoloration, and on imaging the tumor extended close to the urinary bladder ( 1 ). [nature.com]

  カサバッハ・メリット現象） K．Capillary malformations（CM：毛細血管奇形） L．Venous malformations（VM：静脈奇形） M．Arteriovenous malformations（AVM：動静脈奇形） N．Lymphatic malformations（LM：リンパ管奇形）（嚢胞状リンパ管腫Macrocystic LM， 海綿状リンパ管腫Microcystic LM，限局性リンパ管腫Lymphangioma circumscriptum） O．Primary lymphedema [molcom.jp]

  カサバッハ・メリット現象） K．Capillary malformations（CM：毛細血管奇形） L．Venous malformations（VM：静脈奇形） M．Arteriovenous malformations（AVM：動静脈奇形） N．Lymphatic malformations（LM：リンパ管奇形）（嚢胞状リンパ管腫Macrocystic LM，海綿状リンパ管腫Microcystic LM，限局性リンパ管腫Lymphangioma circumscriptum） O．Primary lymphedema [kinokuniya.co.jp]

  Primary lymphedemas are considered a subtype of LM due to a primary dysgenesis of the lymphatic network. The causal mutations underlying many types of primary lymphedemas are now elucidated (list available on the ISSVA Web site, www.issva.org ). [pediatrics.aappublications.org]

• Fatigue

  Adults often report flu-like symptoms, headache, abdominal pain, fatigue, nausea, and weakness. Interferon response does not seem to correlate with markers of vascular proliferation like urinary basic fibroblast growth factor levels. [dermatologyadvisor.com]

• Fracture

  A, At the age of 29, pathologic fracture of the right humerus ( arrow ); and B, at the age of 32, a bone graft from a vascularized fibular bone gradually disappeared. [nature.com]

  Pathologic fractures may occur in both entities. Primary lymphedemas are considered a subtype of LM due to a primary dysgenesis of the lymphatic network. [pediatrics.aappublications.org]

• Hyperhidrosis

  We found localized hyperhidrosis in two of our patients. Proliferation of eccrine glands near the tumour lobule was a consistent finding in the biopsy specimens from these four patients. [ncbi.nlm.nih.gov]

  […] from 2 to 10cm, most often found on the upper trunk, cervical area, and proximal aspect of the limbs, besides other locations such as face and oral mucosa. 1 Lesions are usually asymptomatic, but can be very painful and present with hypertrichosis and hyperhidrosis [scienceopen.com]

  Hyperhidrosis of the skin overlying the area of the tufted angioma is reported. [emedicine.medscape.com]

  TA is a benign condition, pain and tenderness are common associated symptoms, and hyperhidrosis is a frequent finding that occurs in 30% of patients.[6] In a review of 13 cases of TA, the author observed three different clinical patterns: TA without complications [journals.lww.com]

• Eruptions

  We believe that our case is one of acquired tufted angioma appearing as an eruptive condition similar to eruptive pyogenic granuloma, to which it seems closely related. The involution of lesions in this case is unexplained. [ncbi.nlm.nih.gov]

  One previous case of eruptive tufted angioma has been reported in an immunosuppressed patient. [jcp.bmj.com]

  Eruptive tufted angiomas in a patient with Crohn's disease. Al-Za'abi AM, Ghazarian D, Greenberg GR, Shaw JC. Al-Za'abi AM, et al. J Clin Pathol. 2005 Feb;58(2):214-6. doi: 10.1136/jcp.2004.019018. J Clin Pathol. 2005. [pubmed.ncbi.nlm.nih.gov]

  PMID: 20975337 Eruptive tufted angiomas in a patient with Crohn’s disease. Al-Za’abi AM, Ghazarian D, Greenberg GR, Shaw JC. J Clin Pathol. 2005 Feb;58(2):214-6. PMID: 15677546 [humpath.com]

• Skin Lesion

  lesion References[edit] ^ Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. (2007). [en.wikipedia.org]

  lesion References ↑ Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. (2007). [wikidoc.org]

  She has been regularly followed, and no new skin lesion has since appeared. Figure 2 Patient 4. [nature.com]

  Data collected will include photographs of skin lesions, video images of gastrointestinal lesions, demographic data, clinical information, therapeutic interventions, glass slides of tissue ... [centerwatch.com]

  The nodular lesions varied in color from light to dark red and were restricted to the macules, with no involvement of the normal skin. [edoj.org.eg]

• Hyperpigmentation

  The first biopsy (from the macules) showed basal cell hyperpigmentation, and dermal cellular infiltrate. [edoj.org.eg]

  […] tufted hemangioma have been described, with variable results – in two of them with good response,11,12 and with complete failure in three others.1,13,14 Yet, because it is a relatively safe method, with few side effects, the main being post-inflammatory hyperpigmentation [scielo.br]

  We defined spontaneous regression as the absence of superficial or deep infiltration, even in the presence of residual skin changes, such as hyperpigmentation or telangiectasias.Results We included a total of nine cases, six in females and three in males [analesdepediatria.org]

• Skin Ulcer

  sometimes, extensive skin involvement is seen, but skin ulceration is rare The lesions may be in the form of macules and plaques; sometimes, sclerosing plaques may be seen Pain and increased sweating may be noted In some cases, a nodular tumor mass [dovemed.com]

  ulcers. abstract：:Systemic sclerosis (SSc) is often complicated by severe skin ulcers that are unresponsive to traditional treatments. [shengsci.com]

• Aggressive Behavior

  Our recent results showed that ectopic overexpression of human Prox1 gene, a lymphatic endothelial nuclear transcription factor, promoted an aggressive behavior in 2 murine models of KHE. [ncbi.nlm.nih.gov]

  Tufted angiomas usually have a benign course, with only rare reports of aggressive behavior or local invasion. [1] However, tufted angiomas seldom self-involute. [2] Pathophysiology The pathogenesis of tufted angiomas is not well understood. [emedicine.medscape.com]

  These tumors have a benign course, with no reported cases of aggressive behavior or metastases. In our case report tufted angioma was a deep mass in an adult. This manifestation was never mentioned in the literature till now. [omicsonline.org]

  Thus, the designation of hemangioendothelioma infers to its local aggressive behavior, but no longer for a metastatic potential. [nature.com]

Laboratory tests that may be important in the workup of TA include a basic screen of coagulation factors; particularly complete blood count with platelets, D-dimers, and fibrinogen, but also prothrombin time (PT), partial thromboplastin time and international [dermatologyadvisor.com]

Effective treatments reported in the literature are scarce. We report a case of tufted angioma in a child, which had a successful outcome after treatment with the pulsed-dye laser both in terms of cosmesis and alleviation of pain and discomfort. [ncbi.nlm.nih.gov]

Variable response to propranolol treatment of kaposiform hemangioendothelioma, tufted angioma, and Kasabach-Merritt phenomenon. [scholars.northwestern.edu]

It has been associated with Kasabach-Merritt syndrome, a severe coagulopathy of poor prognosis. There have been very few published cases of oral tufted angiomas, and maxillary bone involvement has not been hitherto reported. [ncbi.nlm.nih.gov]

What is the Prognosis of Tufted Angioma? (Outcomes/Resolutions) The prognosis of Tufted Angiomas with adequate treatment is usually good. [dovemed.com]

The authors describe in-depth the clinical and morphologic aspects of each entity and detail their clinicopathologic correlation, differential diagnosis, prognosis, and therapy. [books.google.com]

Prognosis Although rare cases of spontaneous regression have been reported, the clinical course is often chronic. In some cases, tumours arising in the lower limbs infiltrate and progressively form a hardened (sclerous) tegument. [orpha.net]

Its etiology and pathogenesis are uncertain. Tufted angioma typically occurs during infancy or early childhood and displays various clinical patterns. [ncbi.nlm.nih.gov]

TERMINOLOGY Synonyms Acquired tufted angioma (ATA) Angioblastoma (of Nakagawa) Progressive capillary hemangioma Tufted hemangioma Definitions Multiple cannonball-like, scattered cellular collections of small vessels in dermis ETIOLOGY/PATHOGENESIS Unknown [basicmedicalkey.com]

Etiology Kasabach-Merritt syndrome is usually caused by kaposiform hemangioendothelioma or tufted angioma. Other vascular tumors may be responsible, such as infantile hemangioma. The radiographic features are specific to each vascular tumor. [radiopaedia.org]

The etiology of kaposiform hemangioendothelioma remains unknown. It is not associated with HHV8 infection or Kaposi sarcoma. Tumors often involve soft tissue of extremities as ill-defined, violaceous lesions and may encase normal structures. [path.upmc.edu]

Her main area of interest is cancer epidemiology, with a particular focus on melanoma and other skin cancers. Nagwa M. Elwan is Professor of Dermatology at the Faculty of Medicine, Tanta University, Egypt. [books.google.com]

[…] lobular pattern Considered to be on the same neoplastic spectrum as kaposiform hemangioendothelioma; some consider it to be the same entity Terminology Synonyms: Tufted angioma, Nakagawa’s angioblastoma, progressive capillary hemangioma, tufted hemangioma Epidemiology [pathologyoutlines.com]

Note the characteristic podoplanin / D2-40 stain https://twitter.com/ikletskaya/status/822082053814493184 Epidemiology sporadic or familial (autosomal dominant with reduced penetrance) usually occurs in children younger than 5 years may occur in adults [humpath.com]

Summary Epidemiology The prevalence is unknown but around 200 cases have been described in the literature. [orpha.net]

Tufted angiomas usually have a benign course, with only rare reports of aggressive behavior or local invasion. [1] However, tufted angiomas seldom self-involute. [2] Pathophysiology The pathogenesis of tufted angiomas is not well understood. [emedicine.medscape.com]

Pathophysiology The reasons for and mechanism of vascular proliferation are unclear. Some have postulated that high estrogen levels may promote proliferation of TA (because of cases arising in pregnancy). [dermatologyadvisor.com]

Familiarity with distinctive histologic features of tufted angioma should prevent these lesions from being misdiagnosed as either Kaposi's sarcoma or as angiosarcoma. [ncbi.nlm.nih.gov]

Also, post-operative follow-up care with regular screening and check-ups are advised, especially to monitor for any recurrences How can Tufted Angioma be Prevented? Current medical research has not established a method of preventing Tufted Angioma. [dovemed.com]

It can also be used in conjunction with excision to prevent recurrence and shrink remaining tumor burden. Typical dosing is 0.25mg/kg/weekly for many weeks, depending on response. [dermatologyadvisor.com]

Consequently, we chose radical excision of the lesion to prevent, if the medical treatment was ineffective, the lesion from reaching critical areas. Various options exist for medical treatment. [elsevier.es]

One can also argue whether an early intervention with interferon for example, may prevent complications such as the Kasabach-Merritt syndrome, or the local aggressive infiltration of this tumor. [nature.com]