Chronic Plaques in a Patient With Ataxia Telangiectasia

Murakawa, George J.; McCalmot, Timothy; Frieden, Ilona J.

doi:10.1001/archderm.134.9.1145

Cited by 12 publications

(5 citation statements)

References 22 publications

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“…Included were all primary publications published in English, German and French. Ten primary reports were found describing cutaneous granulomas in A-T [4,5,6,7,8,9,10,11,12,13] and a series of articles reporting skin granulomas in other PIDs [14,15,16,17,18,19,20,21,22,23,24,25,26]. For common variable immunodeficiency (CVID), often associated with granulomas in various organs, only the recently published article of Ardeniz and Cunningham-Rundles [23] dealing with skin granulomas was included.…”

Section: Methodsmentioning

confidence: 99%

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Cutaneous Granulomas in Ataxia Telangiectasia and Other Primary Immunodeficiencies: Reflection of Inappropriate Immune Regulation?

Chiam¹,

Verhagen²,

Haraldsson

et al. 2011

Dermatology

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Background: Non-infective cutaneous granulomas with unknown pathogenesis occur in various primary immunodeficiencies (PIDs) including ataxia telangiectasia (A-T). Objective: To find a common immunological denominator in these cutaneous granulomas. Methods: The dermatological and immunological features of 4 patients with A-T and cutaneous granulomas were described. The literature on skin granulomas in A-T and in other PIDs is reviewed. Results: All 4 A-T patients had progressive granulomas on their limbs and showed decreased IgG and IgA concentrations with normal IgM levels. They had a marked decrease in B cells and naïve T cells coinciding with the appearance of the cutaneous granulomas. Similar B- and T-cell abnormalities were described in patients with other PIDs with skin granulomas. Conclusions: We hypothesize that the pathogenesis of these skin granulomas is related to immune dysregulation of macrophages due to the absence of naïve T cells with an appropriate T-cell receptor repertoire and the unopposed activity of γδ T cells and/or natural killer cells.

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Section: Methodsmentioning

confidence: 99%

“…We traced in the literature 17 reported A-T patients with cutaneous granulomas [4,5,6,7,8,9,10,11,12,13] (table 2). There was a female preponderance (13 females, 4 males) compared with equal sex distribution in A-T patients in general.…”

Section: Review Of the Literaturementioning

confidence: 99%

Cutaneous Granulomas in Ataxia Telangiectasia and Other Primary Immunodeficiencies: Reflection of Inappropriate Immune Regulation?

Chiam¹,

Verhagen²,

Haraldsson

et al. 2011

Dermatology

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“…3) and CaTiO 3 -NdAlO 3 (CTNA) 4,5) solid solution compounds; (ii) ceramics with high " r (>80) and moderate Q Á f ( 10;000 GHz), such as tungsten-bronze-related materials in the BaO-Ln 2 O 3 -TiO 2 system (Ln = rare earth element). [6][7][8] There are currently no commercial near-zerof , high-Q microwave dielectrics with 45 < " r < 80, as illustrated in Fig.…”

Section: Introductionmentioning

confidence: 99%

“…Usually, near-zerof ceramics may be obtained by forming solid solutions from end members with opposite signs of f such as in CaTiO 3 -NdAlO 3 - 4,5) or ZrTiO 4 -ZnNb 2 O 6 12) -based ceramics. Other methods of tuning dielectric properties have also been reported in the literature.…”

Section: Introductionmentioning

confidence: 99%

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Composite Dielectric Ceramics Based on BaO–Ln₂O₃–TiO₂ (Ln = Nd, La)

Zheng

Reaney

Muir

et al. 2005

Jpn. J. Appl. Phys.

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BaLa 4 Ti 4 O 15 (BLT) is a hexagonal perovskite-related compound with a temperature coefficient of resonant frequency ( f ) of À2 ppm/ C, relative permittivity ð" r Þ $ 44 and figure of merit ðQ Á f Þ $ 44000 GHz. Ba 4 Nd 9:333 Ti 18 O 54 (BNT) has a tungsten-bronze-related structure with " r $ 78, Q Á f % 11000 GHz and f of þ47 ppm/ C. The microstructures and microwave dielectric properties of xBNT-(1 À x)BLT (0 x 1) composite ceramics have been studied. X-ray diffraction analysis and scanning electron microscopy revealed that there was limited inter-reaction between the two phases and that samples were composed largely of BNT and BLT, although some deterioration in measured " r with respect to calculated values was observed. The optimum compositions were x ¼ 0:55 and 0.75 for which " r $ 63, f $ À20 ppm/ C and Q Á f > 10;000 GHz.

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