For children less than age 12 years, we recommend laser ﬂuences that generate slight purpura. We like to use a large spot size (10–12 mm in diameter for PDL or the large foot- print of the IPL) to minimize skipped areas. The initial treatment session usually results in an average improve- ment of approximately 50%. Each subsequent treatment provides an additional increment of approximately 10% improvement. After six treatments, 40% of our patients completely clear, and those who are not clear have an average improvement of approximately 80% (Fig. 2.19). We have not had patients with scarring or persistent pig- mentary changes despite rare episodes of vesiculation and crusting after treatment. Skin type (I–III) also does not appear to inﬂuence the ultimate treatment outcome, but darker skin requires more treatment sessions to achieve the same degree of clearing as in PWSs of fair-skinned patients as we recommend lower ﬂuences and a higher degree of epidermal cooling. Lesions on distal limbs respond with less fading than lesions elsewhere, such as on the neck and torso (Table 2.6). The diminished response of PWS on the limbs has been reported by others. 94,95
There is sufficient evidence of efficacy to support the use of PDL therapy for treatment of superficial hemangiomas or the superficial component of mixed hemangiomas, and for post- involutional hemangiomas and telangiectasia in infants or children requiring definitive treatment to alleviate or prevent medical or psychological complications. (Hayes Directory, Pulsed Dye Laser Therapy for CutaneousVascularLesions, 2012) One reasonably well-designed randomized controlled trial evaluating the efficacy of 585 nm PDL in infants found that early PDL treatment of uncomplicated hemangiomas was no better than a wait-and-see policy, and may even increase the risk of skin atrophy and hypopigmentation. (Batta et al., 2002) In contrast, evidence from lesser quality studies suggests that PDL therapy can induce involution, prevent enlargement, or eliminate cutaneous hemangiomas in selected cases. (Chang et al., 2001; Raulin and Greve, 2001; Hohenleutner et al., 2001) None of the deep hemangiomas or the deep components of mixed hemangiomas responded to PDL therapy. The results from randomized controlled trials suggest that the hemangiomas that responded well in other studies may have resolved
be applied to a small subset of patients who develop depres- sion immediately after a stroke. The second theory suggests that an accumulation of small lesions eventually reaches a threshold that can lead to the onset of depression. In other words, this “threshold” theory hypothesizes the possibility of vulnerabilities caused by wide cerebrovascular disturbances, and it would be the most appropriate theory to explain depres- sion in patients with latent neurologic lesions or those who have suffered a stroke. In clinical studies, neurocognitive function tests conducted on the elderly have revealed defi- cits in attention and executive function when the total area of WMH exceeded 10 cm 2 . 108 Such cognitive dysfunction
All patients with acute onset of a focal neurologic deficit admitted to our neurologic emergency unit, where we see an average of about 2000 patients per year, were included in the study. Patients with neu- rologic symptoms due to an etiology other than cerebral ischemia (eg, cerebral hemorrhage, tumor, Todd’s palsy) and patients with contra- indications for MR imaging where excluded. Of the patients receiving DW MR imaging, all 510 with acute hyperintense lesions were in- cluded in this study. All patients received extra- and transcranial col- or-coded duplex sonographic examination, transthoracic echocardi- ography, and 24-hour electrocardiographic monitoring as well as blood and urine laboratory tests. Additional transesophageal echo- cardiography was performed in 73% of all patients, in whom trans- thoracic echocardiography, holter electrocardiography, and duplex sonography of the extra- and intracranial vessels did not show evi- dence for cardiogenic or large-artery embolism. In all cases, stroke risk factors were recorded.
A 38-year-old female presented with systemic features like fever and skin rash with sheet of pustules, vesicles and generalized exfoliation on face, trunk (both back an chest), upper and lower extremities on an erythematous background for 3-4 days duration (Figure 1a, b, c). Lesions began as erythema and pruritic papules appeared on face, followed by folds of all joints and upper back and rapidly progressed peripherally extending to large areas of body. Papules turned to sheet of pustular lesions shortly, that dried and began to exfoliate in next 3-4 days. There was no history of drug intake and the patient was menstruating (3 rd day) at the time of presentation. Patient was treated with oral glucocorticoids and antihistaminic and the lesions subsided shortly. Full blood count and liver function and renal function tests were normal. She was discharged with in few days with a diagnosis of acute generalized exanthematous pustulosis.
documented. However, the underlying mechanisms responsible for this increased risk are poorly understood. Previous studies using rigorous smooth muscle cell (SMC) lineage tracing have shown abundant SMC investment into atherosclerotic lesions, where SMCs contribute to the formation of a protective fibrous cap. Studies herein tested whether radiation impairs protective adaptive SMC responses during vascular disease. To do this, we exposed SMC lineage tracing (Myh11-ER T2 Cre YFP + ) mice to lethal radiation (1,200 cGy) followed by bone marrow transplantation prior to atherosclerosis development or vessel injury. Surprisingly, following irradiation, we observed a complete loss of SMC investment in 100% of brachiocephalic artery (BCA), carotid artery, and aortic arch lesions. Importantly, this was associated with a decrease in multiple indices of atherosclerotic lesion stability within the BCA. Interestingly, we observed anatomic heterogeneity, as SMCs
450 g and placental disc measured 16 cm in greatest diameter and 2 cm in maximal thickness with membranous insertion of umbilical cord. Furthermore it revealed multiple well circum- scribed creamy rubbery masses measuring from 0.2 to 4 cm at maternal surface (Figure 1). Microscopic examination of dif- ferent nodules showed well demarcated lesions composed of numerous capillary sized vascular channels that were covered by trophoblastic layers (Figures 2 – 4).
level. Definitive correlations between specific histopath- ologic features and clinical disease have not been dem- onstrated to date. Hanno and Mana have reported that specific skin lesions usually have no prognostic signifi- cance, do not show any correlation with the extent of systemic involvement and do not indicate a more ser- ious form of sarcoidosis [9,12]. Our findings contradict the results of these studies, but the the sample size of our study is much greater than that in the others. However, Olive has noted that patients with skin lesions are more likely to have systemic involvement which supports our results . The frequency of cutaneous in- volvement and particular types of skin lesions of sar- coidosis vary across different races or regions [14,15]. In addition to sarcoidosis being more frequent in individ- uals of Afro-American origin, skin involvement in these individuals may also have a more severe form . Ethnic differences may play an important role for skin and systemic involvement as well as progressive disease. Our patient population included had a very wide ethnic base and it was almost impossible to identify the ethnic origin. They were followed up at least for five years after the initial diagnosis and are currently under control. The minimum six years follow-up is a sufficient period of time for a medical prognosis of sarcoidosis in regard to cutaneous involvement. Our findings are statistically significant but they may not be clinically significant. Further studies with larger populations are needed to discriminate clinical and prognostic differences between patients with specific and nonspecific cutaneouslesions in sarcoidosis.
Abstract: Reported herein is a medical curiosities vascular tumor primary arising from the kidney and exhibiting unique histopathological features. A 32-year-old woman underwent a total nephrectomy of right kidney because of a mass localized in the inferior pole. Distinct from other vascularlesions, on histology the tumor had a peculiar composite pattern, consisting of benign and malignant vascular components, which were haphazardly intermixed without any definite margins. The malignant component was composed of epithelioid hemangioendothelioma (45%) and angiosarcoma (50%) with moderate differentiation. Immunohistochemically, the oval to cuboidal to spindle tumor cells expressed only endothelial markers (CD31, CD34 and factor VIII-related antigen). And the angiosarco- matous component was characterized by the presence of a greater proliferation index Ki-67. Unlike other epithelial tumors, smooth muscle actin (SMA), cytokeratin, EMA and S-100 were all negative in the epithelioid tumor cells. These findings led to the diagnosis of a low-grade vascular neoplasm with morphological features consistent with so-called composite hemangioendothelioma (CHE). At 11 month follow up the patient was alive, without evidence of tumor recurrence. CHE is an extremely rare vascular neoplasm, with borderline malignant potential, which mostly occurs in distal extremity of the limbs at the cutaneous level and, only 30 cases have been previously described until now. To our knowledge, this is the first report of CHE arising from the kidney and widens the spectrum of primary vascular tumors arising in the kidney.
Abstract: Objective: To investigate clinical characteristics and treatment effect of sarcoidosis with cutaneouslesions in Chinese patients, and to compare them with previous works. Methods: Retrospective analysis was conducted based on clinical manifestations, systemic examinations and treatment of biopsy-proved 36 patients with sarcoid- osis with cutaneouslesions in our hospital since 2000. Patients were divided into cutaneous sarcoidosis (CS) group without systemic involvement and systemic sarcoidosis (SS) group with systemic involvement according to whether extracutaneous systems were involved. Results: Male to female ratio was 1:4.1 in total 36 patients. Average age of onset was (43.6±15.8) years old in CS group and (54.4±11.5) years old in SS group. The most common cutaneouslesions were papulonodules (41.7%) and frequently found in limbs (61.1%). There were 26 patients in SS group, and lung was the most common organ with systemic involvement, followed by lymph nodes. In SS group, elevation of in- flammatory parameters and evident changes of chest radiologic examination were often observed. 72.2% patients were treated with glucocorticoid and the overall therapeutic efficacy rate was 48.4%. The therapeutic efficacy in CS group (80%) was significantly higher than SS group (33.3%). Papulonodules type had better response to therapy and usually resolved after treatment. Lupus pernio type was resistant to treatment. Conclusion: Sarcoidosis occurs more frequently in females. Lung is the most commonly affected extracutaneous organ in SS patients. CS patients have better response to therapy than SS patients. Types of cutaneouslesions and existence of systemic involvement are related to prognosis of cutaneouslesions.
Brucellosis is the most common zoonotic bacterial infection worldwide with endemic patterns in some countries such as Iran, Turkey, Kuwait, Saudi Arabia, and Peru (most commonly seen in the Middle East, Central, and South America). 1–3 The disease is caused by Brucella species; a small, gram-negative, non-motile, non-spore-forming, intracellular-reproducing, aerobic coccobacilli microorganism that can survive inside macrophages due to speci ﬁ c survival mechanisms. 1,4 Dairy products contaminated with Brucella melitensis (mostly sheep and goat milk) seems to be the ﬁ rst main source of human brucellosis worldwide. 5 Brucellosis is a systemic disease and systemic infection may involve any organ or system of the body such as reticuloendothelial system (spleen, liver, bone marrow, and lymph nodes), gastrointestinal, central nervous system, cardiovascular, musculoskeletal, genitourinary, and integumentary systems. 6,7 Cutaneous involvement has also been described for brucellosis, but it is uncommon and unspeci ﬁ c. 8 Manifestations of cutaneous involvement have been described mostly as disseminated papulonodular eruption, erythema nodosum-like lesions, diffuse maculopapular rash, maculopapular eruptions, psoriasiform lesions, palmar erythema, malar eruption, and palmar eczema. 9–11 In summary, we can divide skin lesions associated with brucellosis into most frequent, such as papulonodular
fast bacilli (AFB). She had lived in Canada for most of her adult life aside from yearly, extended vacations in Ari- zona, USA. She was not aware of any contact with tuber- culous individuals, and had no history of exposure to fish tanks. She recalled experiencing minor trauma to her right wrist just prior to the emergence of the nodular lesions. A Mantoux test was not performed. Physical examination revealed tender nodular and erythematous ankle and wrist lesions (3 cm and 1 cm in diameter, respectively), with full thickness skin ulceration. Chest X-ray was nor- mal. While awaiting culture results, she was started on clarithromycin 500 mg orally twice daily, ciprofloxacin 500 mg orally twice daily, and rifabutin 300 mg orally once daily, after which her wrist lesion slowly healed. The ankle lesion did not respond significantly to medical ther- apy. X-rays demonstrated osteolysis of the distal tibia con- sistent with osteomyelitis. She developed gastrointestinal intolerance to ciprofloxacin and rifabutin, and was con- tinued on clarithromycin alone. At this time, her poly- cythemia treatment was subsequently switched from busulfan to hydroxyurea. The wrist and ankle bone biopsy specimens were inoculated onto plain and hemin-supple- mented Middlebrook 7H10 agar media and BACTEC 12B blood culture bottles incubated at 30°C in an aerobic atmosphere (National Mycobacteriology Laboratory, Edmonton, Canada). After 1 and 6 weeks, respectively, growth of acid-fast bacilli was observed only on the hemin-supplemented Middlebrook slants inoculated with the wrist and ankle specimens and definitively iden- tified as M. haemophilum by high-performance liquid chromatography (Laboratoire de sante publique du Que- bec, Montreal, Canada). Susceptibility testing was per- formed using Etest (AB BIODISK, Solna, Sweden) on Mueller-Hinton agar supplemented with sheep blood. The organism was susceptible to clarithromycin (MIC ≤ 16 µg/ml) and rifabutin (MIC ≤ 0.12 µg/ml) but resistant
Noonan syndrome and NF1 are among the most com- monly encountered germline alterations, with an incidence of 1 in 2,500—1 in 3,000 of the population. Noonan syn- drome, a complex clinical genetic disorder, is inherited as an autosomal dominant trait caused by alterations in PTPN11, SOS1, RAF1, KRAS, NRAS, and BRAF genes and characterized by short stature, craniofacial dysmorphism, short neck with webbing, deformity of the sternum, cardiac and clotting anomalies, and cryptorchidism . The most common germline alterations involve PTPN11 (*50 %), SOS1 (10–15 %), and RAF1 (5–10 %); with KRAS muta- tions only occurring in 2 % of those affected. The severity and the spectrum of the phenotypic changes are significant, with some individuals having almost no clinical stigmata of the disease. A small proportion of individuals with Noonan syndrome also exhibit multiple gnathic GCG previously reported as Noonan-like/multiple GCG, but this phenotype is now recognized to be allelic with Noonan syndrome , as mutations in PTPN11, SOS1, and RAF1 have been reported in this syndrome [33, 34]. LEOPARD syndrome is also allelic with Noonan syndrome and is associated with two recurrent PTPN11 mutations in exons 7 (Tyr279Cys) and 12 (Thr468Met), although other less common altera- tions are also seen. We have reported on an individual with LEOPARD syndrome and multiple GCG of the jaw caused by alterations in PTPN11 [35, 36]. Very occasional patients with craniofacial cutaneous syndrome, classified as a RASopathy (vide infra), and multiple giant cell tumors of the jaw syndrome have also been reported with BRAF or MAP2K1 mutations .
Despite the striking similarities in the immunopathological features of the experimental model of BP and the human dis- ease, there is one conspicuous difference. The inflammatory infiltrate of early lesions in human BP shows large numbers of eosinophils (1), while in the mouse model, neutrophils are the predominant inflammatory cell type in the perilesional sites (12). The time course study uncovered no signs of eosinophil recruitment into the injection site of the mice up to 24 h after passive transfer of pathogenic anti-mBP180 IgG, while exten- sive subepidermal blistering was seen by 12 h after injection. Therefore, it is quite clear that in experimental BP, eosinophils do not play an important role in the initial stages of subepider- mal blistering. In fact, neutrophil-predominant forms of BP have been described in which eosinophils are absent in the inflammatory cell infiltrate (37). These patients suggest that eosinophils are not a requirement for blister development. It is worth noting that most patient biopsies are obtained . 24 h af- ter the initial onset of disease activity. It is possible that if the mouse model were maintained long term that eosinophils might be recruited in a more chronic phase. While at this point we cannot rule out the possibility that there are critical differences in the pathogenic mechanisms of human BP and the BP mouse model, it remains quite possible, given the available informa- tion, that eosinophils are not directly involved, or play only a secondary role, in the initiation of human BP. These cells may well be important in later stages of the disease, e.g., wound healing after tissue damage.
sporadically although familial cases have autosomal dominant inheritance.(49) They can be localized or extensive. Usually lesions occurring in extremities are localized or segmental and those of head and neck region are extensive than their initial presentation.(21) VM can occur not only in skin and subcutaneous tissue but also in internal organs, bones, skeletal muscle etc. They present as bluish soft compressible masses and increase in size with crying or other Valsalva maneuvers.(52) No thrill or bruit is observed in VM. Their main locations are the head and neck (40%), extremities (40%) and trunk (20%).(64) Complications of venous malformations are not uncommon. Pain localized to the lesion is the most common complication. It occurs mostly over the lesions of extremities which are thrombosed. Phleboliths occur as the consequence of calcification of the thrombosis in malformed venous channels.(65) Abnormal coagulation profiles occur due to localised intravascular coagulation (LIC), frequently described in venous malformations. It is associated with large or extensive, deep VM and palpable phleboliths.(66) Venous malformations of head and neck may cause mechanical obstruction or compression of various vital structures depending on their location and extent. Cosmetic disfigurement is the other psychosocial complication related to VM of head and neck.(67)
DOI: 10.4236/ijohns.2018.75028 269 Int. J. Otolaryngology and Head & Neck Surgery greater than 0.5 mm in diameter (46.84%) & were clinically significant. 127 perforators (57.20%) were radially distributed & 95 perforators (42.79%) had ulnar distribution. 90 perforators (40.54%) were identified on distal side (Radial styloid) & 132 perforators (59.45%) were identified on proximal side (Lateral epicondyle). Mean number of perforators, on radial side was 10.6 & 7.9 on ulnar side; comparison of both using student t paired test gives a P value of 0.006, which is significant. Comparison of mean number of perfora- tors on the distal side was 7.5 & proximal side was 11.0; Student Paired t test gives a P value of 0.003, which was statistically significant. Comparison of mean diameter of perforators on Distal side (1.11) & Proximal side (0.86), side using Student Paired t test gives a P value of 0.01 which was statistically significant. A chi square test was done to compare mean diameter of perfora- tors on distal side, which was more than 1 mm (80%) & less than 1 mm (20%) & on proximal side more than 1 mm (35.6%) & less than 1 mm (64.4%). Chi square value of 42.406 was obtained, degree of freedom value was 1 & P value of <0.001 was achieved which was found to be highly significant. Methylene blue injections demonstrated clusters both in proximal & distal forearm & also marked the cutaneous territory of flap. Three-dimensional computed tomo- graphic angiography reveals a network of linking vessels found to communicate between adjacent perforators & running parallel to radial artery. A total of 15 patients were reconstructed with shape modified radial forearm flap following oncologic resections. Wound healing in all 15 patients was good, with scar as- sessment faring better than traditional radial forearm flap. There was no sen- sory or motor nerve deficit. Although pedicle length was comparatively shorter in shape-modified flap, there was no problem in anastomosing to neck vessels. Conclusion: Increase in knowledge of vascular territory of radial artery per- forators with regards to numbers, size, location, & cutaneous territory can lead to expanded use of radial forearm flap based on either distal or proximal perforator alone. Shape modified technique for harvesting radial forearm flap allows primary closure of donor site. Donor site is better healed and shows a predicted pattern, which is functionally and aesthetically good.